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Clinical guidelines for the management of patients with transposition of the great arteries with intact ventricular septum

The Task Force on Transposition of the Great Arteries of the European Association for Cardio-Thoracic Surgery (EACTS) and the Association for European Paediatric and Congenital Cardiology (AEPC)

Published online by Cambridge University Press:  02 March 2017

George E. Sarris*
Affiliation:
(Greece)
Christian Balmer
Affiliation:
(Switzerland)
Pipina Bonou
Affiliation:
(Greece)
Juan V. Comas
Affiliation:
(Spain)
Eduardo da Cruz
Affiliation:
(USA)
Luca Di Chiara
Affiliation:
(Italy)
Roberto M. Di Donato
Affiliation:
(United Arab Emirates)
José Fragata
Affiliation:
(Portugal)
Tuula Eero Jokinen
Affiliation:
(Finland)
George Kirvassilis
Affiliation:
(USA)
Irene Lytrivi
Affiliation:
(USA)
Milan Milojevic
Affiliation:
(Netherlands)
Gurleen Sharland
Affiliation:
(UK)
Matthias Siepe
Affiliation:
(Germany)
Joerg Stein
Affiliation:
(Austria)
Emanuela Valsangiacomo Büchel
Affiliation:
(Switzerland)
Pascal R. Vouhé
Affiliation:
(France)
*
Corresponding author. Athens Heart Surgery Institute, Leoforos Kifissias 2, Amaroussion, 15125 Athens, Greece. Tel: +30-6932-601020; fax: +30-210-6836013; e-mail: [email protected] (G.E. Sarris).
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Abstract

Type
Original Articles
Copyright
© Cambridge University Press 2017 

1. Preamble

Guidelines summarize and evaluate all available evidence with the aim of assisting physicians in selecting the best management strategy for an individual patient suffering from a given condition, taking into account the impact on outcome and the risk–benefit ratio of diagnostic or therapeutic means. Guidelines are no substitutes for textbooks, primary literature sources, or clinical evaluation and judgment. Guidelines and recommendations should help physicians to make decisions in their daily practice. However, the ultimate specific decisions regarding the care of an individual patient must be made by his/her responsible physician(s).

A large number of guidelines have been issued in recent years by many societies and organizations. Because of the impact of guidelines on clinical practice, quality criteria for their development have been established to make the formulation of guidelines transparent to the user.

We followed the recommendations for formulating guidelines issued by the European Association for Cardio-Thoracic Surgery (EACTS) [Reference Sousa-Uva, Head, Thielmann, Cardillo, Benedetto and Czerny1].

Members of this Committee were selected by the EACTS Congenital Domain and the Association for European Paediatric and Congenital Cardiology (AEPC) to represent all specialities involved with the medical and surgical care of patients with transposition of the great arteries (TGA). The following complex transposition cases remain outside the scope of this article: TGA with aortic coarctation or arch hypoplasia, TGA with ventricular septal defect (VSD) with or without left ventricular outflow tract (LVOT) obstruction, TGA or malposition of the great arteries associated with double-outlet right ventricle or in anatomical or functionally univentricular hearts, and congenitally corrected transposition. In brief, experts in the field were selected and undertook a comprehensive review of the published evidence for management of the various clinically important aspects of this common congenital cardiac anomaly. A critical evaluation of diagnostic and therapeutic procedures was performed. The level of evidence and the strength of recommendation of particular management options were weighed and graded according to predefined scales, as depicted in Tables 1 and 2.

Table 1 Classes of recommendations

Table 2 Levels of evidence

A uniform wording of the stated recommendations to reflect the strength of evidence has been used, in accordance with EACTS policy, as recently published [Reference Sousa-Uva, Head, Thielmann, Cardillo, Benedetto and Czerny1].

Disclosure: The members of the Task Force have provided disclosure statements of all relationships that might be perceived as real or potential sources of conflicts of interest. These disclosure forms are kept on file at the headquarters of the EACTS/AEPC. The Committee report received its entire financial support from the EACTS and AEPC, without any involvement of the pharmaceutical, device or surgical industries.

The Task Force selected by the EACTS and AEPC is responsible for the endorsement process of these joint guidelines. The finalized document has been approved by all the experts involved in the Committee.

The document was revised, and finally approved, by both the EACTS and the AEPC and subsequently submitted for publication simultaneously to the European Journal of Cardio-Thoracic Surgery and Cardiology in the Young.

Limitations: Practice guidelines are to be evidence based, but, in the field of congenital heart disease, most studies involve relatively small patient numbers for any given condition, especially when variants and coexisting lesions are considered. Therefore, there is a paucity of robust data such as prospective randomized trials; consequently, it is frequently impossible to use categories for strength of endorsement that have been used in guidelines documents pertaining to other disciplines. Thus, the vast majority of recommendations in this document are based on expert consensus (level of evidence C) rather than on solid data (level of evidence A or B).

2. Background

Transposition of the great arteries is the most common cyanotic congenital heart defect [Reference Marek, Tomek, Skovranek, Povysilova and Samanek2]. It accounts for approximately 5% of congenital heart disease cases and is characterized by ventriculo-arterial discordance: the left ventricle gives rise to the pulmonary artery and the right ventricle, to the aorta. There is atrioventricular concordance. If no significant additional cardiac lesions are present, it is referred to as TGA with intact ventricular septum (TGA IVS). The lesion is categorized as complex TGA when it has associated cardiac anomalies including VSD (which occurs in up to 45% of cases), LVOT obstruction (25%) and coarctation of the aorta (5%). In general, TGA is not familial. There is no known association with syndromes or chromosomal abnormalities. There is a 2:1 male preponderance.

The anatomical configuration of this anomaly establishes a potentially fatal parallel circulation that results in deep hypoxaemia from lack of mixing, with resulting lactic acidosis and demise. Prompt, adequate preoperative intervention and stabilization, followed by surgical repair and expert postoperative management, favour an excellent outcome, with short-term survival probability around 97–100% in selected centres [Reference Fricke, D’Udekem, Richardson, Thuys, Dronavalli and Ramsay3Reference Stoica, Carpenter, Campbell, Mitchell, da Cruz and Ivy7]. The arterial switch operation (ASO), first described by Adib Jatene in 1976 [Reference Jatene, Fontes, Paulista, Souza, Neger and Galantier8], is currently the procedure of choice when the anatomical conditions and the timeline are appropriate; it is performed in the first month of life. Other alternatives, such as the atrial switch and the two-stage ASO, are reserved for the specific scenarios discussed below. Despite the medical and surgical advances in the management of TGA and the low mortality rate, patients require expert diagnostic evaluation, preferably prenatally, and meticulous multidisciplinary management in the perinatal period, preoperatively, intraoperatively and postoperatively.

3. Diagnosis

3.1 Prenatal diagnosis

3.1.1 Prenatal detection

The diagnosis of TGA can be made accurately before birth if the foetal heart is screened at the time of the obstetric anomaly scan. Some studies have shown that the type of repair likely to be required after birth can be well predicted [Reference Bonnet, Coltri, Butera, Fermont, Le Bidois and Kachaner9Reference Sivanandam, Glickstein, Printz, Allan, Altmann and Solowiejczyk12]. Due to the fact that the previously frequently used four-chamber view in TGA IVS shows no abnormality, the overall proportion of cases of TGA in foetal series has been low compared with postnatal series [Reference Allan, Sharland, Milburn, Lockhart, Groves and Anderson13Reference Jaeggi, Sholler, Jones and Cooper15]. The inclusion of the outflow-tract views at the time of the obstetric foetal anomaly scan results in significant improvement in prenatal detection of the transposition [Reference Sharland16,Reference Wigton, Sabbagha, Tamura, Cohen, Minogue and Strasburger17]. Recent publications have reported improved prenatal detection rates for TGA of up to 50% [Reference Marek, Tomek, Skovranek, Povysilova and Samanek2, Reference Khoshnood, Lelong, Houyel, Thieulin, Jouannic and Magnier18Reference van Velzen, Haak, Reijnders, Rijlaarsdam, Bax and Pajkrt20]. It is now generally more widely recommended that, in addition to the four-chamber view, the views of the cardiac outflow tracts also be included as part of the obstetric screening scan [2123]. A formal programme for education and training regarding the foetal heart is required as part of this process, to ensure that sonographers are taught and can maintain the skills of foetal heart examination [Reference Hunter, Heads, Wyllie and Robson24Reference Tegnander and Eik-Nes28].

3.1.2 Counselling following prenatal detection

If transposition is detected or even suspected from the obstetric anomaly scan, referral should be made to a specialist who is experienced in the diagnosis and management of congenital heart disease in the foetus. This referral should be made as soon as possible after detection of a possible transposition, to have the diagnosis confirmed and to allow the parents to be counselled appropriately [29, Reference Allan, Dangel, Fesslova, Marek, Mellander and Oberhansli30].

Following the diagnosis of TGA, the parents need to be informed of the diagnosis, associations, further management during pregnancy and birth, management after birth and the prognosis for their baby. They also need to be made aware of features that may complicate the management. The parents should be given all the information regarding their baby’s heart condition in a way that they understand and be allowed sufficient time to ask questions. Written information and drawings illustrating the problem should be provided. The parents should be given the opportunity to speak with a paediatric cardiac surgeon as well as having the option to speak with other parents who have had a child with transposition. Contact details of parent support groups, both locally and nationally, can be provided to help them.

3.1.3 Further prenatal management

Because many forms of congenital heart disease are associated with extracardiac abnormalities, including karyotype abnormalities, foetal karyotyping is generally recommended after prenatal diagnosis [Reference Copel, Pilu and Kleinman31Reference Tennstedt, Chaoui, Korner and Dietel34]. However, cases of TGA are rarely associated with chromosomal anomalies. It is important to liaise with foetal medicine specialists in order to exclude any associated extracardiac abnormalities. Foetal karyotyping is not generally indicated or recommended in TGA IVS, but the option of karyotyping can be discussed on an individual basis. Following the initial diagnosis and counselling, further foetal cardiology assessment will be required later in the pregnancy. The number and timing of further scans may vary depending on local practices, but they should include an assessment in the few weeks prior to delivery to look for high-risk features [Reference Jouannic, Gavard, Fermont, Le Bidois, Parat and Vouhé35Reference Punn and Silverman37].

a Class of recommendation.

b Level of evidence.

Recommendations for prenatal detection

3.2 Perinatal management—timing, place and mode of delivery

Studies comparing the outcome of babies with TGA diagnosed prenatally with those diagnosed postnatally suggest that the rates of preoperative and postoperative mortality [Reference Bonnet, Coltri, Butera, Fermont, Le Bidois and Kachaner9, Reference van Velzen, Haak, Reijnders, Rijlaarsdam, Bax and Pajkrt20, Reference Fuchs, Muller, Abdul-Khaliq, Harder, Dudenhausen and Henrich38, Reference Khoshnood, De Vigan, Vodovar, Goujard, Lhomme and Bonnet39] and morbidity [Reference Escobar-Diaz, Freud, Bueno, Brown, Friedman and Schidlow19, Reference Bartlett, Wypij, Bellinger, Rappaport, Heffner and Jonas40Reference Verheijen, Lisowski, Stoutenbeek, Hitchcock, Bennink and Meijboom43] are lower for babies diagnosed prenatally.

3.2.1 Site and timing of delivery

Because babies with TGA require early treatment after birth, it is generally recommended that delivery takes place at or near a tertiary-care paediatric cardiology and paediatric cardiac surgery centre [Reference Hellstrom-Westas, Hanseus, Jogi, Lundstrom and Svenningsen44, Reference Mirlesse, Cruz, Le Bidois, Diallo, Fermont and Kieffer45]. Adhering to this practice enables the neonate to be in optimal condition and avoids neonatal retrieval transport-related complications and costs [Reference Jegatheeswaran, Oliveira, Batsos, Moon-Grady, Silverman and Hornberger46]. Although the delivery must be scheduled before the due date, the majority of women can have a vaginal delivery, which is generally recommended [Reference Landis, Levey, Levasseur, Glickstein, Kleinman and Simpson47]. However, a planned caesarean delivery may be indicated if high-risk maternal or foetal features are identified.

a Class of recommendation.

b Level of evidence.

Recommendations for perinatal management

3.3 Postnatal diagnosis

3.3.1 Postnatal detection

The newborn with TGA and inadequate intercirculatory mixing will be symptomatic from birth. Severe cyanosis is an early, almost universal clinical finding, which at least during the first hours after birth, may be the only sign. Screening for arterial oxygen saturation (SaO2) is indicated for early identification of initially asymptomatic patients with TGA, when the pre- or post-ductal value or both are <95% [Reference Eckersley, Sadler, Parry, Finucane and Gentles48].

3.3.2 Further diagnostic steps

Once cyanotic congenital heart disease is suspected, transthoracic echocardiography should be performed immediately, because duration of deep cyanosis and tissue hypoxia are important additional factors in determining ventricular function, acidosis and eventually multiple organ failure.

The results observed on chest radiographs can be normal, but the following abnormal features can also be observed: oval or egg-on-side cardiac shape (due to the narrow mediastinum), mild cardiomegaly and increased pulmonary vascular markings. The electrocardiogram (ECG) may be normal, with the typical neonatal findings of right-axis deviation and right ventricular hypertrophy. Echocardiography is the modality of choice for a definitive diagnosis.

At the time of echocardiography, one should pay particular attention to the root of the great arteries and to the coronary arteries or concomitant features such as VSD, LVOT obstruction, coarctation and mitral valve anomalies. In particular, the diameters of the main pulmonary artery and the aorta have to be measured; the location of the valve commissures and also the origin and course of the coronary arteries must be described carefully before surgery.

It has been shown that echocardiography facilitates accurate evaluation of the coronary artery pattern and exclusion of other relevant malformations [Reference McMahon, el Said, Feltes, Watrin, Hess and Fraser49, Reference Pasquini, Sanders, Parness, Wernovsky, Mayer and Van der Velde50]. In addition, echocardiography facilitates imaging for the safe performance of balloon atrial septostomy (BAS) (also known as the Rashkind procedure). Because BAS can be safely performed under echocardiographic guidance, preoperative diagnostic cardiac catheterization should be considered only in selected cases for diagnosis of complex lesions or if institutional experience does not permit performance of atrial septostomy under echocardiographic guidance.

BAS: balloon atrial septostomy; TGA: transposition of the great arteries.

a Class of recommendation.

b Level of evidence.

c References

Recommendations for postnatal diagnosis

4. Perinatal management

Significant regional differences exist in the organization of care for newborns with TGA. In most instances, especially in the absence of prenatal diagnosis, the newborn with TGA will need to be stabilized in a neonatal intensive care unit (ICU) and subsequently transported to a tertiary-care centre, where definitive surgical care is available. Although elective intubation of infants on prostaglandin E1 (PGE1) prior to transport has been common practice in many institutions, several studies have shown that the rate of complications is significantly higher in infants who need intubation [Reference Browning Carmo, Barr, West, Hopper, White and Badawi55, Reference Meckler and Lowe56]. Occasionally, BAS may be available locally and may be performed prior to transport.

4.1 Monitoring and immediate care

Preoperative monitoring of patients with TGA in the ICU includes mostly noninvasive technologies associated with the clinical evaluation of vital signs and peripheral perfusion and cardiovascular examination (although invasive strategies may be required): pre- and post-ductal pulse oximetry, continuous ECG, noninvasive blood pressure monitoring, respiratory rate and pattern monitoring. Inspired end-tidal capnography may be used and reserved for ventilated patients. In addition to vital signs, urine output must be monitored closely, but the insertion of a Foley catheter is not justified unless the patient is haemodynamically compromised. Tissue perfusion monitoring may be followed by serial testing for blood lactate levels and near-infrared spectroscopy (NIRS) in the decompensated phase. Neonates with TGA might have umbilical venous, and eventually umbilical arterial, lines inserted promptly after birth, which facilitates safe administration of drugs, including—but not exclusively limited to—PGE1; surveillance of invasive haemodynamic parameters, as needed; fluid administration and acid–base follow-up and management. The use of other central lines should be minimized in the preoperative period unless the patient remains critically ill. To assess mixed venous saturations, sampling in the innominate vein is required to avoid overestimates because of the atrial level mixing. Fluids ought to be administered without restrictions, and the indications do not vary with standard neonatal recommendations.

4.2 Haemodynamic management

The initial management of newborns with TGA should focus on stabilization, optimization of mixing of systemic and pulmonary circulations (management of hypoxia) and oxygen delivery, maintenance of adequate systemic perfusion and correction of acidosis.

The immediate priority after birth and throughout the first few hours of life is to determine whether the mixing between systemic and pulmonary circulations is adequate. Immediately after birth, an intravenous (IV) infusion of PGE1 is recommended to maintain ductal patency until the comprehensive series of postnatal echocardiograms is complete and all sites of intercirculatory mixing have been evaluated. PGE1 has been used in various dosing regimens: a higher dose of up to 0.1 µg/kg/min may be necessary when the ductus needs to be reopened. To maintain patency, starting doses vary from 0.0125 to 0.05 µg/kg/min, and patients can be weaned, starting 2–4 h following initiation, provided that oxygen (O2) saturations and tissue perfusion remain acceptable. Nevertheless, the use of PGE1 may not suffice, because ductal shunting is often inadequate in the presence of a restrictive interatrial communication. These patients warrant an emergent atrial septostomy. Throughout the performance of the atrial septostomy, or in those patients needing longer-term ventilation, sedation and analgesia are occasionally required. The usual combination of drugs includes nonopioids (i.e. paracetamol), opioids (low-dose morphine or fentanyl) and benzodiazepines. Dexmedetomidine has emerged as a useful and safe drug with anxiolytic properties and no significant respiratory depressive effect [Reference Barton, Munoz, Morell and Chrysostomou57].

Patients presenting with deep hypoxaemia, acidosis and in shock must benefit from the emergent measures recommended in neonatal advanced life-support algorithms. Concomitantly, an infusion of PGE1 should be emergently started at high doses (0.1 µg/kg/min) while preparing for the atrial septostomy.

Once adequate mixing has been achieved at the atrial level, discontinuation of PGE1 is often possible, unless there is an associated left-heart obstruction (i.e. coarctation of the aorta). Notwithstanding this attempt, successful discontinuation of the drug is unpredictable [Reference Finan, Mak, Bismilla and McNamara58, Reference Oxenius, Hug, Dodge-Khatami, Cavigelli-Brunner, Bauersfeld and Balmer59]. Because of the risk of rebound hypoxaemia after abrupt discontinuation of PGE1, it is recommended that, after septostomy, patients should be weaned from PGE1 rather than stopped. Patients remaining on PGE1 must be observed for potential side and adverse effects. The risk of apnoea may be attenuated by the administration of caffeine or with stimulation tools like a high-flow nasal cannula or continuous positive airway pressure [Reference Lim, Kulik, Kim, Charpie, Crowley and Maher60]. Furthermore, persistent left-to-right shunting across the ductus arteriosus may cause pulmonary oedema, which may affect patient stability and require escalation of therapy and airway support. Pragmatically, it may be adequate to adopt a permissive attitude with regards to the degree of cyanosis rather than exposing patients to the deleterious effects of excessive blood flow to maintain a higher arterial O2 saturation level.

Further haemodynamic measures to support decompensated patients include colloids or crystalloids for volume expansion, use of O2 and correction of metabolic acidosis.

A few neonates may remain significantly cyanotic and acidotic even after the atrial septostomy. In such circumstances, echocardiography should be performed to confirm the unrestrictive nature of the atrial septal defect (ASD) as well as of the patent ductus arteriosus and to determine the presence and degree of pulmonary hypertension. The diagnosis of pulmonary hypertension is usually confirmed using echocardiography. Although cut-off values are difficult to define, the rate of diagnosed pulmonary hypertension varies in the available literature. The incidence of persistent pulmonary hypertension in neonates with TGA is 12.5%, and it occurs more frequently in cases of TGA IVS [Reference Roofthooft, Bergman, Waterbolk, Ebels, Bartelds and Berger61, Reference Newfeld, Paul, Muster and Idriss62].

Mortality is high in this group of neonates and mid-term postoperative outcomes are negatively affected [Reference Roofthooft, Bergman, Waterbolk, Ebels, Bartelds and Berger61, Reference Fan, Hu, Zheng, Li, Zhang and Pan63]. Given that it is a serious condition with a high mortality rate, different treatment strategies have been used with variable success, including sedation, paralysis and hyperventilation [Reference Chang, Wernovsky, Kulik, Jonas and Wessel64], inhaled nitric oxide (NO) [Reference El-Segaier, Hellstrom-Westas and Wettrell65], sildenafil, bosentan [Reference Goissen, Ghyselen, Tourneux, Krim, Storme and Bou66] and extracorporeal life support (ECLS), alone or in combination [Reference Jaillard, Belli, Rakza, Larrue, Magnenant and Rey67, Reference Luciani, Chang and Starnes68]. Because the existing literature consists mainly of case reports, management should include the stepwise introduction of the above-mentioned treatment modalities and close monitoring of the clinical response (improved oxygenation). Such patients may require the resumption of PGE1 because the ductus arteriosus may be useful as a ‘pop-off’ and ultimately improve systemic tissue perfusion.

4.3 Mechanical ventilation and respiratory measures

Systemic SaO2 saturation in TGA depends on the relative proportions and O2 saturation levels of the two sources of the systemic circulation, i.e. fully saturated pulmonary venous blood that is shunted from the pulmonary to the systemic circulation (‘effective’ systemic flow) and the systemic mixed venous blood that recirculates through the systemic vascular bed. The degree of intercirculatory mixing is dictated by the number, size and site of anatomical communications between the two circuits. The haemoglobin level is also important, and a level of around 15 g/dl is considered optimal. Systemic and pulmonary vascular resistances (PVR) add to the complex interplay of the preceding factors [Reference Mair and Ritter69, Reference Shaher70]. Preoperative manipulation of mixing and the other contributing factors should result in an O2 saturation level of 75–85% of the arterial blood gas. In preterm newborns, the lower end of the acceptable range can be as low as 70%. One important point is that the accuracy of pulse oximetry values <80% is limited in neonates [Reference Shiao and Ou71] and frequent monitoring of arterial blood gases may be warranted.

Neonates with profound hypoxaemia (partial pressure of arterial oxygen <25 mmHg and/or SaO2 <60%) require urgent attention [Reference Jouannic, Gavard, Fermont, Le Bidois, Parat and Vouhé35].

Conservative measures to increase systemic O2 saturation levels and adequate tissue oxygen delivery include (i) continuous PGE1 infusion to maintain ductal patency and emergent BAS to increase intercirculatory mixing; (ii) mild hyperventilation and increased fraction of inspired oxygen (FiO2) to lower PVR and increase pulmonary blood flow; (iii) transfusion to treat relative anaemia and increase O2-carrying capacity; (iv) sedation and paralysis to decrease O2 consumption; and (v) possibly inotropic support to increase cardiac output and O2 delivery [Reference Marino, Bird and Wernovsky72].

4.4 Nutrition

Infants with TGA and adequate intercirculatory mixing, without PGE1 infusion (e.g. after septostomy), should be fed enterally and encouraged to bottle-feed and breast-feed [Reference Howley, Kaufman, Wymore, Thureen, Magouirk and McNair73Reference Sables-Baus, Kaufman, Cook and da Cruz75]. There is still great controversy surrounding the best approach to enteral nutrition for infants with cyanotic congenital heart defects, especially during the time when they are prostaglandin-dependent [Reference Howley, Kaufman, Wymore, Thureen, Magouirk and McNair73]. Based on limited evidence in favour or against the practice of feeding infants enterally while they are on PGE1 [Reference Willis, Thureen, Kaufman, Wymore, Skillman and da Cruz74, Reference Davis, Davis, Cotman, Worley, Londrico and Kenny76, Reference Natarajan, Reddy Anne and Aggarwal77], haemodynamically stable newborns with TGA should be fed enterally as soon as it is deemed feasible preoperatively, even while they are on PGE1. Breast milk and breast-feeding are preferred. Trophic enteral feeding may be considered in some patients in order to reduce the risk of translocation.

4.5 Major prematurity and very low birth weight

The incidence of low birth weight among newborns with TGA is reported to be 3.05% [Reference Soongswang, Adatia, Newman, Smallhorn, Williams and Freedom78], which compares favourably with the reported 15% overall incidence of prematurity or low birth weight in neonates with congenital heart disease [Reference Ades, Johnson and Berger79]. Although it is clear that low birth weight and prematurity are different factors, they often coexist. Low birth weight (≤2.5 kg), very low birth weight (≤1.5 kg) and, less so, prematurity [Reference Hickey, Nosikova, Zhang, Caldarone, Benson and Redington80] present technical and physiological challenges to complete repair in the neonate. Additional comorbidities from other organ systems (central nervous system, renal, gastrointestinal) increase the morbidity and mortality rates of these infants both short and long term [Reference Reddy81]. More specifically, in transposition, large, multi-institutional studies in Europe and North America have demonstrated increased mortality rates after an ASO in infants weighing <2.5 kg [Reference Curzon, Milford-Beland, Li, O’brien, Jacobs and Jacobs82, Reference Kansy, Tobota, Maruszewski and Maruszewski83]. However, it has been shown that delaying repair to allow for weight gain confers higher preoperative morbidity and early mortality without any associated benefit [Reference Reddy, McElhinney, Sagrado, Parry, Teitel and Hanley84, Reference Roussin, Belli, Bruniaux, Demontoux, Touchot and Planche85]. Furthermore, delaying intervention for TGA IVS results in deconditioning of the left ventricle, rendering the patient a potentially poor candidate for a primary ASO. Centres have reported early repair [Reference Azakie, Johnson, Anagnostopoulos, Egrie, Lavrsen and Sapru86], primary repair as late as age 3 months, late single-stage repair with postoperative mechanical circulatory support and two-stage repair (i.e. pulmonary artery banding with or without aortopulmonary shunt placement followed by an ASO in 7–14 days) [Reference Azakie, Johnson, Anagnostopoulos, Egrie, Lavrsen and Sapru86, Reference Rios, Dummer and Overman87] with acceptable results.

ASO: arterial switch operation; ECLS: extracorporeal life support; IV: intravenous; PGE1: prostaglandin E1; VAD: ventricular assist device.

a Class of recommendation.

b Level of evidence.

Recommendations for perinatal management in a neonatal intensive care unit

5. Surgery for Transposition of The Great Arteries With Intact Ventricular Septum

5.1 Timing for surgery of TGA IVS

The ASO for TGA IVS in newborns was introduced in the early 1980s. The assumption was that the neonatal left ventricle would be suited for systemic work after having withstood systemic pressure throughout foetal life [Reference Castaneda, Norwood, Jonas, Colon, Sanders and Lang88, Reference Quaegebeur, Rohmer, Ottenkamp, Buis, Kirklin and Blackstone89]. The neonatal ASO has since become the preferred approach for repair of TGA IVS and is currently achievable with an average surgical mortality rate of 2–5% [Reference Sarris, Chatzis, Giannopoulos, Kirvassilis, Berggren and Hazekamp90].

At birth, the left ventricular muscle mass is equivalent to that of the right ventricle. Subsequently, as a result of the rapid postnatal decrease in PVR, the left ventricle soon becomes ‘deconditioned’, losing muscle mass and the ability to function at systemic workloads [Reference Danford, Huhta and Gutgesell91, Reference Rudolph92]. Currently, the optimal timing for an ASO in babies with TGA IVS is established from the first few days to 3 weeks of life [Reference Duncan, Poirier, Mee, Drummond-Webb, Qureshi and Mesia93].

In Europe, 25–30% of patients with TGA IVS have undergone a routine ASO within the first week of life [Reference Sarris, Chatzis, Giannopoulos, Kirvassilis, Berggren and Hazekamp90]. It is worth noting that an ASO in the first few hours of life may obviate the need for BAS [Reference Chasovskyi, Fedevych, Vorobiova, Zhovnir, Maksimenko and Boychenko94, Reference Nevvazhay, Chernogrivov, Biryukov, Biktasheva, Karchevskaya and Sulejmanov95]. This very early approach, however, remains controversial.

Also, the upper age limit for a primary ASO in TGA IVS cannot be determined. Most surgeons undertake a primary ASO in babies up to 4 weeks of age, whereas the choice of a primary ASO beyond 1 month of age is controversial [Reference Sarris, Chatzis, Giannopoulos, Kirvassilis, Berggren and Hazekamp90]. In fact, several groups have electively adopted a primary ASO in late presenters (up to 8 weeks of age), planning postoperative mechanical support, if necessary [Reference Duncan, Poirier, Mee, Drummond-Webb, Qureshi and Mesia93, Reference Bisoi, Sharma, Chauhan, Reddy, Das and Saxena96Reference Foran, Sullivan, Elliott and de Leval99], and accepting prolonged duration of postoperative ventilation and hospital stay [Reference Kang, de Leval, Elliott, Tsang, Kocyildirim and Sehic100]. A few outliers undergoing an ASO at up to 9 months of age have been reported [Reference Kang, de Leval, Elliott, Tsang, Kocyildirim and Sehic100, Reference D’Udekem, Cheung, Butt, Shann and Brizard101]. However, for infants older than 2 months, left ventricular mass and mass/end-diastolic volume ratio should, preferably, orient towards a rapid two-stage ASO.

ASO: arterial switch operation; ECLS: extracorporeal life support; IVS: intact ventricular septum; TGA: transposition of the great arteries.

a Class of recommendation.

b Level of evidence.

c References.

Recommendations for timing of the ASO

5.2 Adequacy of left ventricular myocardial mass

5.2.1 Left ventricular mass regression

Upon completion of a postnatal fall of pulmonary arteriolar resistance (around the fourth week of life), left ventricular mass in TGA IVS starts decaying [Reference Rudolph92, Reference Di Donato, Fujii, Jonas and Castaneda102], although with some degree of reversibility [Reference Bisoi, Malankar, Chauhan, Das, Ray and Das103, Reference Rudolph104].

In addition, isolated pulmonary outflow obstruction in TGA IVS, whether anatomical [Reference Bisoi, Sharma, Chauhan, Reddy, Das and Saxena96] or dynamic [Reference Lacour-Gayet, Piot, Zoghbi, Serraf, Gruber and Mace105, Reference Robinson, Wyse and Macartney106], may trigger left ventricular myocardial hypertrophy and potentially allow an ASO to be performed. Furthermore, a moderately restrictive ASD and a sizeable (≥5 mm) patent arterial duct may both preserve adequate left ventricular preload and pressure and partly explain the positive outcomes in some late presenters [Reference Kang, de Leval, Elliott, Tsang, Kocyildirim and Sehic100]. Finally, genetically predetermined factors might also account for the involution of PVR and left ventricular performance [Reference Bisoi, Sharma, Chauhan, Reddy, Das and Saxena96].

5.2.2 Assessment of left ventricular preparedness

Left ventricular ‘preparedness’ for an ASO is commonly judged on measurable parameters (e.g. left ventricular geometry, wall thickness and function on echocardiogram) and on additional evidence of pressure and volume loading related to the size of the duct and an ASD [Reference Danford, Huhta and Gutgesell91, Reference Kang, de Leval, Elliott, Tsang, Kocyildirim and Sehic100, Reference Lacour-Gayet, Piot, Zoghbi, Serraf, Gruber and Mace105, Reference Bano-Rodrigo, Quero-Jimenez, Moreno-Granado and Gamallo-Amat107Reference Smith, Wilkinson, Arnold, Dickinson and Anderson110]. The ventricular septum is forged by unequal ventricular pressures and progressively shifts towards the pulmonary ventricle, assuming a banana-shaped appearance on an echocardiogram [Reference Iyer, Sharma, Kumar, Bhan, Kothari and Saxena111, Reference Sidi, Planche, Kachaner, Bruniaux, Villain and Le Bidois112]. The Marie Lannelongue group introduced echo-based markers to judge preparedness [Reference Lacour-Gayet, Piot, Zoghbi, Serraf, Gruber and Mace105]. On the contrary, the Great Ormond Street group found that, in the late ASO group, conventional measures of left ventricular pressure and function were not predictive of mortality or of the need for mechanical support [Reference Foran, Sullivan, Elliott and de Leval99]. Alternatively, left ventricular preparedness may be assessed by a ‘provocative’ pulmonary artery banding: If tolerated by the left ventricle for up to 15–30 min, a primary ASO is undertaken [Reference Dabritz, Engelhardt, von Bernuth and Messmer113].

5.3 Training of the left ventricle for a delayed arterial switch operation

5.3.1 Introduction and pathophysiological issues

In 1977, Yacoub et al. [Reference Yacoub, Radley-Smith and Maclaurin114] devised a two-stage approach for an ASO in older patients with TGA IVS that included a preparatory pulmonary artery banding together with a systemic-to-pulmonary shunt for left ventricular training, followed by an ASO several months later. However, this policy did not become widely adopted for frequent, intractable, postoperative, left ventricular dysfunction, probably because of the advanced age at pulmonary banding [Reference Borow, Arensman, Webb, Radley-Smith and Yacoub115, Reference Sievers, Lange, Onnasch, Radley-Smith, Yacoub and Heintzen116]. In 1989, Jonas et al. [Reference Jonas, Giglia, Sanders, Wernovsky, Nadal-Ginard and Mayer117] introduced the so-called rapid two-stage ASO, showing that left ventricular hypertrophy is elicited as early as 1–2 weeks after the imposition of a pressure load in younger patients beyond neonatal age.

An 85% increase in left ventricular mass within 5–7 days of applying a pulmonary artery band was demonstrated in infants with TGA IVS [Reference Jonas, Giglia, Sanders, Wernovsky, Nadal-Ginard and Mayer117, Reference Boutin, Jonas, Sanders, Wernovsky, Mone and Colan118]. Remarkably, both the capacity and the rapidity of left ventricular hypertrophy decrease with aging. The age limit at which the potential for myocyte hyperplasia in the human infant is lost is allegedly 3–6 months after birth [Reference Di Donato, Fujii, Jonas and Castaneda102].

5.3.2 Indications for a two-stage arterial switch operation

Categorical indications for left ventricular training include a combination of the following noninvasive criteria:

  1. 1. Indexed left ventricular mass <35 g/m2.

  2. 2. Age well above 3 weeks.

  3. 3. Ventricular septal profile, with a banana-like left ventricular shape on 2D echocardiograms.

  4. 4. Absence of a patent arterial duct or LVOT obstruction [Reference Lacour-Gayet, Piot, Zoghbi, Serraf, Gruber and Mace105].

Haemodynamic data may also be used, especially if BAS is achieved using heart catheterization rather than 2D echocardiographic guidance. Aortic and systemic venous oxygen saturations, right atrial pressure and the left/right ventricular pressure ratio may then be obtained [Reference Wernovsky, Giglia, Jonas, Mone, Colan and Wessel119]. In general, a left/right ventricular pressure ratio <0.6 is an indication for a staged ASO [Reference Dabritz, Engelhardt, von Bernuth and Messmer113, Reference Parker, Zuhdi, Kouatli and Baslaim120].

5.3.3 Types of left ventricular training and technical aspects
5.3.3.1 Hypoxic (pre-ASO) left ventricular training.

Hypoxic left ventricular training, often preceded by BAS, implies a two-stage ASO with preliminary imposition of either pressure or volume overload or, more commonly, combinations of both. Whichever of the three methods described below is used, a tolerable level of systemic O2 saturation and an adequate left ventricular preload must be sought.

  1. 1. Pulmonary artery banding combined with a systemic–pulmonary shunt (usually a modified Blalock–Taussig anastomosis) followed by an ASO after an interval that depends on the patient’s age [i.e. 1–2 weeks in young infants (rapid two-stage ASO) [Reference Lacour-Gayet, Piot, Zoghbi, Serraf, Gruber and Mace105, Reference Jonas, Giglia, Sanders, Wernovsky, Nadal-Ginard and Mayer117], or several months in older infants/children] [Reference Yacoub, Radley-Smith and Maclaurin114, Reference Parker, Zuhdi, Kouatli and Baslaim120Reference Helvind, McCarthy, Imamura, Prieto, Sarris and Drummond-Webb122]. A moderate degree of both pressure and volume overload provides the most effective stimulus for ventricular hypertrophy, and a small-to-moderate ASD is advantageous to ensure the necessary volume preload for the left ventricle [Reference Ilbawi, Idriss, DeLeon, Muster, Gidding and Duffy123]. Through either a sternotomy or a thoracotomy, a systemic–pulmonary shunt is placed first, using a polytetrafluoroethylene (PTFE) vascular graft (size 3.5 mm). After opening the shunt, under an FiO2 of 30%, the pulmonary artery band is placed and, while directly monitoring the left ventricular pressure, sequentially tightened to obtain a left/right ventricular systolic pressure ratio of 0.7. Simultaneous 2D echocardiographic guidance provides information on the tightness of the banding: the occurrence of ventricular failure suggests that the banding is too tight. Postoperatively, patients are weaned from inotropes and mechanical ventilation, based upon echocardiographic documentation of sustained good left ventricular function [Reference Lacour-Gayet, Piot, Zoghbi, Serraf, Gruber and Mace105, Reference Helvind, McCarthy, Imamura, Prieto, Sarris and Drummond-Webb122]. In older patients, sequential tightening over several months may be necessary [Reference Bernhard, Yacoub, Regensburger, Sievers, Smith and Stephan124]. In the presence of a wide ASD for intercirculatory mixing, the single best predictor of SaO2 is the magnitude of pulmonary blood flow. Acute reduction of total pulmonary blood flow, as happens following pulmonary artery banding, drastically cuts both effective pulmonary and systemic flows [Reference Mair and Ritter69]. If no additional source of pulmonary blood flow is contemplated, the degree of banding must be mild enough to allow sufficient effective pulmonary blood flow. In these cases, a slower myocardial hypertrophic response should be expected.

  2. 2. Pulmonary artery banding combined with induced patency of the arterial duct, obtained either by prostaglandin infusion or by ductal stenting, depending on the anticipated duration of the interim period.

  3. 3. Induced patency of the arterial duct alone, obtained using either prostaglandin infusion or ductal stenting [Reference Sivakumar, Francis, Krishnan and Shahani125]. In this case it may be advisable NOT to pursue a wide ASD, to assure adequate preload of the left ventricle [Reference Harinck, Van Mill, Ross and Brom126]. Simple ductal stenting, or supposedly prolonged prostaglandin infusion, may also rapidly train the involuted left ventricle of late presenters within days to a few weeks [Reference Foran, Sullivan, Elliott and de Leval99, Reference Ilbawi, Idriss, DeLeon, Muster, Gidding and Duffy123, Reference Harinck, Van Mill, Ross and Brom126]. It can be a less morbid method of left ventricular training because it avoids haemodynamic stress, pulmonary artery distortion and neoaortic valve regurgitation. The use of moderate-sized (3.5 or 4 mm) coronary stents was suggested to avoid post-procedure heart failure [Reference Sivakumar, Francis, Krishnan and Shahani125].

5.3.3.2 Normoxic (post-ASO) left ventricular training.

Normoxic left ventricular training is adopted after an ASO presenting intraoperative left ventricular failure unrelated to a coronary problem. It may be achieved pharmacologically or by mechanical circulatory support (see section 6.5.1).

5.3.4 Results of left ventricular training for a delayed arterial switch operation

The reported risk of mortality after Stage I is very low and easily avoided by emergency takedown of the pulmonary artery banding [Reference Lacour-Gayet, Piot, Zoghbi, Serraf, Gruber and Mace105, Reference Iyer, Sharma, Kumar, Bhan, Kothari and Saxena111]. The initial postoperative course of these patients, however, is often characterized by significant morbidity associated with low-output syndrome of variable severity and significant metabolic acidosis [Reference Boutin, Wernovsky, Sanders, Jonas, Castaneda and Colan127Reference Wernovsky, Wypij, Jonas, Mayer, Hanley and Hickey129]. A less tight pulmonary banding, with a left/right ventricular peak pressure ratio at 0.65, prevents left ventricular dysfunction while endorsing ventricular remodelling [Reference Lacour-Gayet, Piot, Zoghbi, Serraf, Gruber and Mace105, Reference Ilbawi, Idriss, DeLeon, Muster, Gidding and Duffy123].

The left/right ventricular pressure ratio increases from 0.5 before pulmonary banding to 1.0 before the ASO. Most of the increase in left ventricular mass (95%) occurs in the first week, with the most rapid rate of hypertrophy by Day 2 and an exponential fall in the growth rate thereafter. The left ventricular volume also progressively increases, but not as rapidly as the left ventricular mass, with a consequent gradual rise in left ventricular mass/volume ratio without acute dilation. The left ventricular ejection fraction is significantly reduced at 12 h after banding but returns to basal levels by 3.5 days after banding as compensatory hypertrophy takes place [Reference Iyer, Sharma, Kumar, Bhan, Kothari and Saxena111, Reference Boutin, Jonas, Sanders, Wernovsky, Mone and Colan118].

The early mortality rate after a rapid two-stage ASO is between 0 and 6%, and the postoperative course can be smoother than in a single-stage ASO due to the excellent left ventricular mass developed [Reference Lacour-Gayet, Piot, Zoghbi, Serraf, Gruber and Mace105, Reference Iyer, Sharma, Kumar, Bhan, Kothari and Saxena111, Reference Jonas, Giglia, Sanders, Wernovsky, Nadal-Ginard and Mayer117, Reference Ilbawi, Idriss, DeLeon, Muster, Gidding and Duffy123]. The late follow-up of the two-stage approach has revealed impaired left ventricular systolic performance [Reference Boutin, Wernovsky, Sanders, Jonas, Castaneda and Colan127], increased incidence of neoaortic regurgitation [Reference Colan, Boutin, Castaneda and Wernovsky128] and right ventricular outflow tract (RVOT) obstruction [Reference Wernovsky, Giglia, Jonas, Mone, Colan and Wessel119]. Nonetheless, most of these patients enjoy an excellent clinical condition and physical ability [Reference Lacour-Gayet, Piot, Zoghbi, Serraf, Gruber and Mace105].

5.3.5 Optimal time interval between stages

The key tool for surgical decision making after Stage I is 2D echocardiography [Reference Lacour-Gayet, Piot, Zoghbi, Serraf, Gruber and Mace105, Reference Iyer, Sharma, Kumar, Bhan, Kothari and Saxena111, Reference Jonas, Giglia, Sanders, Wernovsky, Nadal-Ginard and Mayer117]. Clinical and haemodynamic parameters are also important [Reference Wernovsky, Wypij, Jonas, Mayer, Hanley and Hickey129]. Proposed criteria for a safe second-stage ASO include left-to-right ventricular pressure ratio >0.85, left ventricular end-diastolic volume >90% of normal, left ventricular ejection fraction >0.5, posterior wall thickness >4 mm and a predictive wall stress <120×103 dynes/cm2 [Reference Nakazawa, Oyama, Imai, Nojima, Aotsuka and Satomi130]. At Marie Lannelongue, the ASO was performed when the left ventricular mass had reached 50 g/m2 [Reference Lacour-Gayet, Piot, Zoghbi, Serraf, Gruber and Mace105].

The median interval between Stages I and II is 10 days (range 5 days to 6 weeks) [Reference Lacour-Gayet, Piot, Zoghbi, Serraf, Gruber and Mace105, Reference Iyer, Sharma, Kumar, Bhan, Kothari and Saxena111, Reference Boutin, Jonas, Sanders, Wernovsky, Mone and Colan118]. Provided that adequate left ventricular mass and volume are rapidly achieved, the early Stage II ASO has the advantage of avoiding pericardial adhesions.

ASO: arterial switch operation; BAS: balloon atrial septostomy; IVS: intact ventricular septum; PTFE: polytetrafluoroethylene; TGA: transposition of the great arteries.

a Class of recommendation.

b Level of evidence.

c References.

Recommendations for left ventricular training

5.4 Surgical techniques and intraoperative surgical management

5.4.1 Intraoperative parameters and cardiopulmonary bypass

Cardiopulmonary bypass (CPB) policy regarding temperature, flow, the use of vasodilators and pH status management varies widely without a clear consensus among the members of the surgical community and with little evidence to justify endorsing one approach over another. Compared with low flow bypass, a deep hypothermic circulatory arrest (DHCA) strategy in infancy is associated with worse neuro-developmental outcomes. DHCA should, therefore, be avoided whenever possible, due to both early and late unfavourable impacts [Reference Bellinger, Wypij, duPlessis, Rappaport, Jonas and Wernovsky131, Reference Karl, Hall, Ford, Kelly, Brizard and Mee132].

Cannulation for CPB varies according to preference. The ascending aorta is typically cannulated just proximally to the innominate artery, and venous return occurs through a single atrial basket. Direct or indirect bicaval cannulation is a valid alternative, making intracardiac procedures more flexible without circulatory arrest. No evidence favours any of the methods. A properly placed systemic vent enhances visibility.

Myocardial protection strategies vary widely, from cold crystalloid to multidose cold blood cardioplegia, used antegradely through the aortic root and then directly through the coronary ostia. Recently, warm bypass strategies and warm blood cardioplegia were introduced with claimed advantages; however, none of the myocardial protection methods has yet been shown to be preferable.

Weaning off bypass should be straightforward unless there is ischaemia (related to any coronary transfer occurrence), left ventricular dysfunction due to ischaemia or ventricle detraining. Less commonly, problems with RVOT reconstruction or transient pulmonary hypertension may create weaning difficulties but are easily recognized.

Immediate postoperative targets are a stable ECG, with no electrical instability (suggesting ischaemia), left atrial pressure 5–15 mmHg, with evidence of good tissue perfusion and urine output >1 ml/kg/h. High lactate levels and ongoing acidosis are poor prognostic markers and require active corrective measures [Reference Charpie, Dekeon, Goldberg, Mosca, Bove and Kulik133, Reference Munoz, Laussen, Palacio, Zienko, Piercey and Wessel134].

There is no direct evidence to suggest that routine use of milrinone (a phosphodiesterase inhibitor) following an ASO improves outcome but it is recommended from extrapolation to its use for other infant cardiac procedures. It is recommended that milrinone be started for any patient with signs or symptoms of low cardiac output and with at least a left atrial pressure >15 mmHg. The recommended loading dose of milrinone is 50 µg/kg over 30–60 min, followed by an infusion of 0.375–0.750 µg/kg/min. If hypotension develops, blood pressure support with other inotropic/vasopressor agents (epinephrine or dopamine) may be necessary [Reference Hoffman, Wernovsky, Atz, Kulik, Nelson and Chang135].

Bleeding is a well-known problem after an ASO in neonates. Perfect suture lines are essential to prevent bleeding, but the use of antifibrinolytic drugs is common. Formerly, aprotinin in different doses and regimens proved to be effective and safe for kidney function [Reference Bojan, Vicca, Boulat, Gioanni and Pouard136]. Both epsilon-aminocaproic acid and tranexamic acid were introduced; tranexamic acid has the same level of safety and similar level of efficacy as epsilon-aminocaproic acid and is associated with improved outcomes. The use of prophylactic antifibrinolytic agents for ASOs should be considered [Reference Pasquali, Li, He, Jacobs, O’brien and Hall137]. The use of recombinant factor VII (rVII) was recently introduced and might be a valuable addition in cases with severe bleeding. However, scientific proof for the use of rVII is lacking.

Acute renal failure is prevalent after an ASO. However, prophylactic use of a peritoneal dialysis catheter is not recommended [Reference Bellinger, Wypij, duPlessis, Rappaport, Jonas and Wernovsky131].

Delayed sternal closure after an ASO has been a routine technique for many surgical groups over the years. Studies do not support the hypothesis that elective delayed sternal closure will reduce the morbidity after an ASO in neonates but they do confirm the safety and efficacy of the procedure.

5.4.2 Coronary transfer

All coronary artery patterns are theoretically transferable [Reference Quaegebeur, Rohmer, Ottenkamp, Buis, Kirklin and Blackstone89, Reference Kirklin, Blackstone, Tchervenkov and Castaneda138]. Coronary artery patterns have been identified as a risk factor for mortality in ASOs [Reference Sarris, Chatzis, Giannopoulos, Kirvassilis, Berggren and Hazekamp90, Reference Prêtre, Tamisier, Bonhoeffer, Mauriat, Pouard and Sidi139Reference Wong, Finucane, Kerr, O’donnell, West and Gentles146]. Different techniques have been used for coronary transfer: the button technique in which coronary ostia are transferred to punch holes, and tissue is removed from the neoaortic root, to accommodate the ostia; the slit technique in which slit openings that may be linear or U-shaped are created in the neoaorta; and the trap-door technique in which L-shaped incisions are created, leaving a hinged flap, with an angle that seems to favour coronary transfer and improves landing. None of these techniques was demonstrated as being superior [Reference Bove147, Reference Villafane, Lantin-Hermoso, Bhatt, Tweddell, Geva and Nathan148]; however, the trap-door technique is recognized for reducing angulation for all cases, particularly for double-loop patterns.

In some specific cases, pericardium hoods allow for more flexibility and safer transfer [Reference Parry, Thurm and Hanley149, Reference Tireli, Korkut and Basaran150].

Large coronary buttons should be harvested, often requiring removal of most of the respective sinus of Valsalva. For eccentrically situated ostia, the nearest aortic valve commissure may need to be taken down and later resuspended in the neopulmonary root. The proximal segments of the coronary artery should be mobilized adequately to allow kink-free, tension-free and torsion-free translocation.

The coronary ostia are to be transferred to the respective facing sinuses, preferably laterally, and should not be compressed by the facing neopulmonary trunk, upon distension. The location on the sinuses is dictated by anatomical details, namely commissural placement. In most cases, surgeons prefer to place the left coronary button rather low, whereas the right coronary button needs to be placed higher. In some cases, namely posteriorly looping patterns, the right coronary artery button in pattern D is better implanted into a higher position, above the sinotubular junction and the main vessel suture line.

A single-ostium coronary pattern is a specific transfer challenge. It may or may not be associated with an intramural course of the proximal coronary artery, which may involve a commissural area. The two standardized techniques are the Yacoub technique [Reference Yacoub and Radley-Smith151] and the Imai technique [Reference Koshiyama, Nagashima, Matsumura, Hiramatsu, Nakanishi and Yamazaki152]. In the Yacoub technique, the single coronary ostium is harvested with a large aortic cuff, mobilizing the adjacent commissure if necessary. The ostium is then anastomosed to the posteriorly facing neoaorta, along its superior border, the pouch being completed by the distal end of the aorta, which is cut obliquely, leaving a long anterior lip. Alternatively, a pericardial patch can be used to augment the aortic suture line to accommodate the pouch. In this case, the ostium is not rotated more than 90°, allowing no torsion. This method of transfer is applied to a single-ostium Yacoub type B classification and also to a Yacoub type C in which the two coronary ostia are very close to each other and truly impossible to split apart. Alternatively, the Imai technique can be used: the ostium, or closely lying ostia, is left untouched without harvesting or without any rotation; the aortic wall above the ostium is excised to within 1–2 mm of the ostium; and a window opening is created to the adjacent neoaortic root. A small semilunar patch of pericardium or adjacent aortic tissue as a rotated flap of the non-coronary sinus is sutured to the inferior edges of the coronary button, creating a wide pouch. The reconstruction is completed by the ascending aortic suture line to the superior end of the pouch [Reference Koshiyama, Nagashima, Matsumura, Hiramatsu, Nakanishi and Yamazaki152]. None of these alternative methods was found to be superior; however, their use is recommended for single or ‘too-close’ ostia transfer [Reference Qamar, Goldberg, Devaney, Bove and Ohye153Reference Mee159].

An intramural course often occurs in the presence of a single ostium but may occur with other complex patterns, also involving one or two main coronary arteries. Whenever it is detected, an intramural course should be addressed by unroofing, even when a commissure is involved. In this case the commissural area must be repaired. After unroofing, the coronary ostia are to be transferred normally using a trap-door technique [Reference Thrupp, Gentles, Kerr and Finucane144, Reference Asou, Karl, Pawade and Mee160, Reference Chen, Cui, Chen, Yang, Cui and Xia161].

Transferring coronary arteries in the presence of non-facing great arteries, i.e. in pure side-by-side vessel arrangements, is extremely challenging. Individualized techniques are recommended, i.e. using extensive proximal coronary artery mobilization, trap doors and even pericardium tube extensions, for anterior loops with distant lateralized vessels. The use of autologous pericardial hoods is sometimes useful to accommodate unexpected anatomical problems.

For experienced surgeons, all coronary artery patterns are said to be transferrable. However, there may be occasions in which the only safe option is to opt for the atrial switch. These cases are exceptional and are not recommended by any level of evidence or recommendation.

5.4.3 Right ventricular outflow tract reconstruction

The reconstruction of the neopulmonary trunk connecting the former aorta to the pulmonary bifurcation depends on the construction of a tension-free anastomosis, in order not to create any coronary compression and trying to minimize the risk of tension-induced late RVOT stenosis. This goal requires several manoeuvres: full mobilization of pulmonary arterial branches as far as the origin of the first pulmonary branching into the lung hilum; reconstruction of pulmonary tissue defects related to excision of the Valsalva sinuses area; and anterior or more lateral pulmonary bifurcation displacement, which is achieved mainly by the Lecompte manoeuvre.

The Lecompte manoeuvre [Reference Lecompte, Zannini, Hazan, Jarreau, Bex and Tu162], by which the pulmonary bifurcation is brought anterior to the aorta, should be performed routinely [Reference Delmo Walter, Miera, Nasseri, Huebler, Alexi-Meskishvili and Berger163Reference Raju, Burkhart, Durham, Eidem, Phillips and Li169] whenever the aorta and pulmonary arterial trunks are orientated totally, or predominantly, anteriorly–posteriorly.

Whenever great vessels are located side by side, and depending on special orientations, the Lecompte manoeuvre may or may not be performed [Reference Chen, Cui, Chen, Yang, Cui and Xia161, Reference Hutter, Kreb, Mantel, Hitchcock, Meijboom and Bennink167, Reference Karl, Cochrane and Brizard170, Reference Lalezari, Bruggemans, Blom and Hazekamp171]. In cases where it is not performed, it is necessary to shift the pulmonary artery bifurcation, which is done by sliding the anastomosis to one of the pulmonary branches.

The pulmonary artery tissue defects related to coronary transfer must be filled in. Several patch materials and patching techniques may be used [Reference Delmo Walter, Miera, Nasseri, Huebler, Alexi-Meskishvili and Berger163, Reference Ullmann, Gorenflo, Bolenz, Sebening, Goetze and Arnold166, Reference Kawata, Kishimoto, Iwai, Ishimaru, Saito and Kayatani168, Reference Karl, Cochrane and Brizard170, Reference Imoto, Kado, Asou, Shiokawa, Miyake and Yasuda172Reference Sakurai, Maeda, Miyahara, Nakayama, Murayama and Hasegawa174]; however, autologous pericardial patch material is used most widely [Reference Delmo Walter, Miera, Nasseri, Huebler, Alexi-Meskishvili and Berger163, Reference Ullmann, Gorenflo, Bolenz, Sebening, Goetze and Arnold166, Reference Sakurai, Maeda, Miyahara, Nakayama, Murayama and Hasegawa174]. Most surgeons use fresh pericardium [Reference Hutter, Kreb, Mantel, Hitchcock, Meijboom and Bennink167, Reference Rudra, Mavroudis, Backer, Kaushal, Russell and Stewart173], because the use of materials pretreated with glutaraldehyde, irrespective of its concentrations, is associated with the development of RVOT stenosis postoperatively [Reference Sakurai, Maeda, Miyahara, Nakayama, Murayama and Hasegawa174].

The so-called trousers patch reconstruction technique is generally used [Reference Delmo Walter, Miera, Nasseri, Huebler, Alexi-Meskishvili and Berger163, Reference Hutter, Kreb, Mantel, Hitchcock, Meijboom and Bennink167, Reference Rudra, Mavroudis, Backer, Kaushal, Russell and Stewart173], because it apparently produces less residual RVOT obstruction [Reference Delmo Walter, Miera, Nasseri, Huebler, Alexi-Meskishvili and Berger163].

In the so-called button hole technique for coronary harvest and transfer, the neopulmonary trunk may reconstructed using direct anastomosis [Reference Swartz, Sena, Atallah-Yunes, Meagher, Cholette and Gensini165, Reference Rudra, Mavroudis, Backer, Kaushal, Russell and Stewart173]; the punch holes should be filled in with patch material.

No evidence has been produced regarding the use of any particular suture material. Resection of a small segment of ascending aorta before aortic reanastomosis in order to allow a more tension-free pulmonary reconstruction has been proposed [Reference Raju, Burkhart, Durham, Eidem, Phillips and Li169].

No evidence regarding the use of glue, systematically or sporadically, to prevent bleeding, is available, although its use has become generalized.

5.4.4 Atrial septal defect in TGA IVS

Patients under consideration for an ASO always have an ASD: a secundum ASD, a patent foramen ovale or an ASD after septostomy. This defect is normally closed, either directly or by patch. As part of the procedure, a residual defect may be left intentionally, to make the postoperative course smoother, particularly in patients in whom PVR might fluctuate and prompt a haemodynamic crisis. However, there is insufficient evidence to make a recommendation in favour or against this practice.

ASO: arterial switch operation; DHCA: deep hypothermic circulatory arrest; ECG: electrocardiogram.

a Class of recommendation.

b Level of evidence.

c References.

Recommendations for management of cardiopulmonary bypass

ASO: arterial switch operation; RVOT: right ventricular outflow tract.

a Class of recommendation.

b Level of evidence.

c References.

Recommendations on surgical technique

6. Perioperative and postoperative management

6.1 Anaesthetic management

Pertinent preoperative and perioperative issues that require special attention include the following:

  1. 1. Cyanosis, which may delay induction of anaesthesia with inhaled anaesthetics: neonates with a high haematocrit and excessive viscosity may have impaired microvascular perfusion, outweighing the advantages of increased oxygen-carrying capacity. Reduction of red blood cell (RBC) volume is not recommended in this situation.

  2. 2. Elevated PVR: special attention must be paid to patients with high PVR who present as ‘poor mixers’ and require urgent surgery with a suboptimal acid–base balance.

  3. 3. Coexisting diseases: these could preclude the use of monitoring options [transoesophageal echocardiogram (TOE)].

  4. 4. Family rapport and parent informed consent: to help the family develop a sense of trust and a positive hospital experience, it is important to discuss with them line placement, prolonged ventilation and instrumentation issues.

6.1.1 Monitoring

Noninvasive monitoring for an ASO should include pulse oximetry (usually two sites, pre- and post-ductal, are used), five-lead ECG, end-tidal capnography, oxygen and anaesthetic gas analysis, automated blood-pressure-measurement cuff, multiple-site (rectal, tympanic or posterior pharyngeal) temperature measurement, volumetric urine collection and a precordial stethoscope during induction. In the presence of cyanosis, pulse oximetry overestimates SaO2 and is exacerbated with further decrease of partial pressure of arterial oxygen (PaO2) [Reference Fanconi176]. The partial pressure of end-tidal CO2 value is less reflective of partial pressure of arterial carbon dioxide (PaCO2) because of ventilation perfusion mismatching [Reference Lazzell and Burrows177]. Rectal and tympanic temperature readings overestimate brain temperature [Reference Stone, Young, Smith, Solomon, Wald and Ostapkovich178]. NIRS has been used routinely to evaluate both cerebral (forehead) [Reference Gottlieb, Fraser, Andropoulos and Diaz179] and tissue (renal) perfusion. Although more data in humans are needed, the use of noninvasive monitoring is likely to improve perioperative management in patients undergoing an ASO [Reference Tortoriello, Stayer, Mott, McKenzie, Fraser and Andropoulos180]. These technologies are useful indicators of trends in oxygenation [Reference Andropoulos, Stayer, Diaz and Ramamoorthy181].

Invasive monitoring includes placement of an arterial line and catheterization of a central vein for central venous pressure (CVP) measurement.

Transoesophageal echocardiogram is an invaluable perioperative tool for monitoring ventricular performance and evaluating surgical results. Attention should be paid to complications that could occur during the use of TOE, especially haemodynamic compromise from left atrial compression.

Blood-gas analysis, along with the ability to perform onsite thromboelastography and platelet count, is as important as haemodynamic monitoring for this procedure.

6.1.2 Induction and maintenance of anaesthesia

No studies support the use of any particular anaesthetic agents for induction and maintenance of anaesthesia. Patients usually present with established IV access. In such cases, an opioid-based anaesthetic is supported. All other IV induction agents could be used at judicious doses, once their primary effects on the myocardium and vascular system are carefully evaluated. In cases of the inhalation-induction technique, administration of sevoflurane, the preferred anaesthetic agent, should be done with care and not in ‘high’ inspired concentrations, which could lead to relative bradycardia and decreased systemic vascular resistance.

All inhaled anaesthetic agents are thought to offer a degree of ischaemic preconditioning not only to the myocardium but also to the brain [Reference Codaccioni, Velly, Moubarik, Bruder, Pisano and Guillet182] and kidney [Reference Lee, Ota-Setlik, Fu, Nasr and Emala183].

Administration of amnesia-inducing agents is frequently minimized, because the importance of perioperative recall in this age group is frequently underestimated. Benzodiazepines given intravenously or a volatile anaesthetic agent administered through a vaporizer on CPB should be considered.

6.2 Pre-cardiopulmonary bypass management

The duration of the pre-CPB period varies and requires the vigilance and intense involvement of the anaesthesiologist, particularly in periods when distractions are unavoidable (such as during line placement). It is important to realize that pre-CPB is the period when the parallel connection of the systemic and pulmonary circulations takes place, whereby adequate mixing is achieved by obtaining appropriate volume status. Systemic oxygenation is highly dependent on increased venous oxygen saturation (SvO2) and improves with volume administration. Some anaesthesiologists have a lower threshold for use of inotropic support during this period, because of its favourable potential impact on cardiac output and therefore oxygenation improvement. There are not enough data to support such a choice. The importance of optimizing PVR and ensuring adequate mixing should not be underestimated. In cases of late TGA corrections, decreased left ventricular performance is also important. Finally, during the pre-CPB period, attention should be paid to careful surgical manipulation of the aorta and vena cava because missteps at this point could precipitate arrhythmias, hypotension and blood loss, leading to further systemic desaturation.

6.3 Cardiopulmonary bypass and anaesthesia

The initiation of CPB introduces major changes in the pharmacokinetics and pharmacodynamics of administered agents. These changes, which are magnified by the volume of distribution changes in the neonate, require additional attention directed towards achieving adequate depth of anaesthesia [Reference Kussman, Zurakowski, Sullivan, McGowan, Davis and Laussen184] and enhancement of the effects of the administered vasoactive agents. Parameters of CPB management that require special anaesthesiological consideration include the following:

  1. 1. Haematocrit and blood product utilization on bypass. Use of blood products for an ASO is unavoidable and practice is institution specific. The majority of centres would add either whole blood or packed RBCs with fresh frozen plasma (FFP) to ensure a haematocrit ≥25%, especially during the cooling and rewarming phases when hypoxic and ischaemic brain injuries are most likely to occur [Reference Newburger, Jonas, Soul, Kussman, Bellinger and Laussen185]. The risk–benefit ratios favour the greater haematocrit approach up to 30%, which represents a shift from previous practice trends [Reference Wypij, Jonas, Bellinger, Del Nido, Mayer and Bacha186]. We propose the use of fresh blood up to 5 days old, because, compared with stored RBCs, fresh RBCs are more metabolically balanced, have a higher pH, contain less potassium and reduced concentrations of lactate [Reference Sumpelmann, Schurholz, Thorns and Hausdorfer187], lead to fewer pulmonary complications and renal dysfunction and have lower infection rates [Reference Mou, Giroir, Molitor-Kirsch, Leonard, Nikaidoh and Nizzi188].

  2. 2. Vascular tone. When an α-stat technique is used, catecholamine production and a relative alkaline environment lead to elevated systemic vascular resistance and not to homogeneous tissue and organ perfusion. The use of vasodilators, mainly α-receptor antagonists, is widely advocated. Phentolamine at 0.1–0.2 mg/kg, phenoxybenzamine, sodium nitroprusside and nitroglycerine offer vasodilating effects.

  3. 3. Systemic inflammatory response. Treatments intended to reduce the systemic inflammatory response to CPB and operative trauma in general include the use of steroids. The use of steroids has been questioned. Although many groups use steroids preoperatively in order to mitigate the inflammatory effects of CPB while providing myocardial protection [Reference Pasquali, Hall, Li, Peterson, Jaggers and Lodge189], various publications show that there are not enough evidence-based data to justify this practice [Reference Gessler, Hohl, Carrel, Pfenninger, Schmid and Baenziger190, Reference Graham, Atz, Butts, Baker, Zyblewski and Deardorff191]. A multicentre observational analysis performed in the USA and published in 2012 did not find any benefit associated with methylprednisolone in neonates undergoing heart surgery and suggested that increased infection occurred in certain subgroups [Reference Pasquali, Li, He, Jacobs, O’brien and Hall192]. Another recent systematic review of randomized controlled trials showed that, despite the demonstrated attenuation of CPB-induced inflammatory response following the administration of steroids and other potential clinical advantages (lower mortality rate and significant reduction of renal-function deterioration), a large prospective randomized study is still needed to verify clearly the effects of steroid prophylaxis in paediatric patients [Reference Scrascia, Rotunno, Guida, Amorese, Polieri and Codazzi193]. Studies of newer anti-inflammatory treatment modalities are under way, including the use of monoclonal antibodies for inflammatory products (i.e. complement, tumour necrosis factor and endotoxins), and these may offer advantages in the future. Currently, adequate heparinization, along with the use of coated circuits [Reference Miyaji, Hannan, Ojito, Jacobs, White and Burke194, Reference Boning, Scheewe, Ivers, Friedrich, Stieh and Freitag195], is recommended, because it offers evidence-based elimination of thrombin production, minimizing the degree of systemic inflammatory reaction [Reference Finley and Greenberg196]. Finally, it should be noted that the use of opioids (fentanyl at doses higher than 25 µg/kg) decreases stress response, modifies inflammation and stabilizes haemodynamics [Reference Hickey, Hansen, Wessel, Lang, Jonas and Elixson197]. The use of conventional and modified ultrafiltration techniques provides evidence of reduction of bypass-related perioperative morbidity. Both improve myocardial function, decrease tissue oedema and minimize ventilatory support times. Modified ultrafiltration does not necessitate the addition of crystalloids or colloids, as does conventional ultrafiltration, uses only the patient’s blood volume, but requires extra vigilance to avoid volume depletion and temperature alterations [Reference Liu, Ji, Long, Li and Feng198].

  4. 4. Glucose control. Although still controversial, glucose control should be kept in mind, to avoid extremes of blood-sugar levels. In the Boston Circulatory Arrest Study, intraoperative [Reference Wypij, Newburger, Rappaport, duPlessis, Jonas and Wernovsky199] and initial postoperative [Reference Ballweg, Wernovsky, Ittenbach, Bernbaum, Gerdes and Gallagher200] hyperglycaemia did not predict worse neuro-developmental outcomes.

6.4 Separation from cardiopulmonary bypass

Issues pertinent to ASOs that play important roles during separation from CPB are as follows:

  1. 1. Myocardial ischaemia resulting from either air emboli or stenotic coronary anastomoses. After the surgical coronary anastomotic result is evaluated, increasing the coronary perfusion pressure should resolve myocardial ischaemia. Elevated RV afterload after the Lecompte manoeuvre could be another cause of coronary ischaemia.

  2. 2. Labile pulmonary artery pressure and elevated PVR that require interventions.

  3. 3. Poor left ventricular performance, especially in late correction of TGA, requiring use of inotropic support (milrinone, epinephrine, dopamine or dobutamine) or mechanical circulatory support.

6.5 Transfer to the intensive care unit

Monitoring of O2 saturation; ECG; and end-tidal carbon dioxide (CO2), arterial, central venous and atrial pressures (if available), should be continuous when the patient is transferred from one area of the hospital to another.

Resuscitation drugs, airway equipment, blood products and fluids for intravascular volume replacement should also be available. Patients are transferred on high FiO2, with a manual resuscitator or preferably a Jackson-Rees circuit, which provides better clinical information for chest and lung compliance. For patients on NO, ensuring continuous administration of NO is required. Various battery charges should be checked as well as pacing availability. Once adequate depth of anaesthesia is assured, the endotracheal tube should be suctioned before arrival in the ICU.

Finally, but most importantly, communication and rapport with the ICU staff should be a top priority well before the patient is moved to the ICU. We recommend that ICU nursing staff be in the operating room before the patient is moved.

CPB: cardiopulmonary bypass; FFP: fresh frozen plasma; NIRS: near-infrared spectroscopy; RBCs: red blood cells.

a Class of recommendation.

b Level of evidence.

c References.

Recommendations for anaesthetic management

6.5.1 Postoperative management in the intensive care unit

Postoperative management of patients with TGA focuses on the optimization of cardiac output [avoidance and treatment of low cardiac output syndrome (LCOS)], tissue perfusion and respiratory status, and on mitigation of the stress response and inflammatory processes triggered by CPB. Early goals of management also include control of coagulopathy; management of vascular tone, anomalies and capillary leak; and prevention and management of total body volume overload.

6.5.1.1 Transition and handover from the operating room

Intensive-care management starts with an adequate, standardized and safe transfer from the operating room. Prior to transfer, a World Health Organization Surgical Safety Checklist should be applied [Reference Bergs, Hellings, Cleemput, Zurel, De Troyer and Van Hiel203]. The objectives are to have a face-to-face handover of information in a systematic, concise, cohesive and safe manner. The anaesthesiologist is responsible for the care of the patient until the report process is complete. The assessment of the patient by the cardiac ICU nurses and physicians must occur after the report process is complete [Reference Boat and Spaeth204Reference Vergales, Addison, Vendittelli, Nicholson, Carver and Stemland209].

6.5.1.2 Haemodynamic and tissue perfusion monitoring

After the ASO, patients require noninvasive and invasive monitoring. Basic monitoring strategies include continuous ECG; respiratory rate; noninvasive blood pressure cuff; core and peripheral temperatures; systemic oximetry and invasive arterial blood pressure; CVP; inspired end-tidal capnography; and, infrequently, continuous left atrial pressure. The acid–base status, mixed venous O2 saturations and serum lactate levels are excellent markers of O2 delivery [Reference Seear, Scarfe and LeBlanc210]. Patients may also be monitored with NIRS, which is a good surrogate marker of mixed venous oxygen saturation and hence a good continuous monitor of cardiac output and O2 delivery [Reference Bhutta, Ford, Parker, Prodhan, Fontenot and Seib211, Reference Mittnacht212]. Alternative technologies include invasive continuous mixed venous saturation monitoring and devices based on arterial wave contour analysis. Urine output must be carefully monitored although it is a poor early indicator of low cardiac output and deficient oxygen delivery.

6.5.1.3 Ancillary evaluation

Baseline evaluation includes a chest radiograph, arterial blood gas with a lactate level, central mixed venous saturation if required (in unstable patients), basic electrolyte and renal function panels and coagulation screening. Patients with bleeding may need more frequent evaluation of coagulation and possibly thromboelastograms, which would direct a goal-orientated therapy. Echocardiography is indicated for all unstable patients. Awareness of signs of coronary insufficiency is of utmost importance. An immediate postoperative ECG is routinely obtained and compared with preoperative evaluations. In the event of electrocardiographic or clinical evidence of ischaemia, nitroglycerine may be started; an echocardiograph, emergency catheterization and immediate surgical revision should be considered.

6.5.1.4 Haemodynamic management

Proper haemodynamic support must be based on comprehensive haemodynamic and pathophysiological appraisals [Reference Holmes213Reference Zaritsky and Chernow215].

The most important take-home message is that the ultimate objective is to provide adequate tissue perfusion. Mean arterial pressures of 35–45 mmHg are acceptable for newborns following an ASO. Left atrial pressures, when monitored, should be maintained in the 8–10 mmHg range because volume boluses to increase this pressure are not well tolerated by the non-compliant left ventricle. The presence of right ventricular hypertension is unusual, and its presence should be promptly investigated using echocardiography. Residual intracardiac shunts or branch pulmonary artery stenosis following the Lecompte manoeuvre have both been implicated in elevated right ventricular pressures [Reference Stoica, Carpenter, Campbell, Mitchell, da Cruz and Ivy7, Reference Deshpande, Wolf, Kim and Kirshbom216]. Neonates undergoing an ASO receive milrinone (0.5–1.0 µg/kg/min) and low-dose dopamine (3–5 µg/kg/min) or low-dose adrenaline (0.01–0.05 µg/kg/min) to augment ventricular function if needed. Calcium chloride (10%) infusions at 5–15 mg/kg/h may also be helpful. Systemic afterload reduction can also be achieved with numerous drugs that add to the vasodilatory effect of milrinone; these may be alpha-blockers (phentolamine, phenoxybenzamine) or sodium nitroprusside. Vascular tone may be an issue in patients who present with significant inflammatory reactions. Vasopressin, low-dose norepinephrine and phenylephrine at low doses are useful to antagonize such vasodilation, optimize target organ perfusion and eventually optimize urine output, while reducing fluid overload [Reference Burton, Kaufman, Goot and da Cruz217].

6.5.1.5 Management of rhythm and conduction disorders

Following an ASO, neonates may be vulnerable to arrhythmias, which may relate to various factors [Reference Decker, McCormack and Cohen218]. Arrhythmias are associated with increased mortality rates; therefore, anticipating risk factors and preventing arrhythmias remain important objectives during the postoperative course [Reference Decker, McCormack and Cohen218, Reference Shamszad, Moore, Ghanayem and Cooper219]. Many arrhythmias are easily solved. In patients undergoing an ASO, indwelling lines may cause arrhythmias triggered by irritation of the myocardium when their tip is in an intracardiac position, and in this case, they require removal [Reference Decker, McCormack and Cohen218, Reference Texter, Kertesz, Friedman and Fenrich220]. Usually automatic tachycardia and re-entry tachycardia may not be well tolerated in the early postoperative phase, particularly in patients with marginal haemodynamics. For patients with arrhythmias, priority should be given to immediate resuscitation efforts if they are haemodynamically unstable; parallel steps focus on the correction of electrolyte and acid–base disturbances, optimization of ventilation, repositioning of intrathoracic lines—general, simple measures that may resolve the arrhythmias. If these measures fail, prompt antiarrhythmic therapy should be initiated. One of the most common arrhythmias after an ASO is junctional ectopic tachycardia (JET). In the management of JET, the following measures are recommended: controlled hypothermia, decrease of vasoactive drugs, optimization of electrolytes, use of dexmedetomidine or amiodarone and pacemaker strategies. Ideally, for safety reasons, antiarrhythmic drugs should be titrated as continuous infusions. Esmolol [221, Reference Trippel, Wiest and Gillette222], procainamide [Reference Benson, Dunnigan, Green, Benditt and Schneider223Reference Mandapati, Byrum, Kavey, Smith, Kveselis and Hannan225] and amiodarone [Reference Dubin224, Reference Coumel and Fidelle226Reference Perry, Knilans, Marlow, Denfield, Fenrich and Friedman229] are the most commonly used antiarrhythmic drugs in the neonatal period [Reference Dubin224]. Other commonly used antiarrhythmic drugs used in the neonate after an ASO are sotalol [Reference Dubin224, Reference Maragnes, Tipple and Fournier230], propranolol [Reference Dubin224], digoxin [Reference Brugada, Blom, Sarquella-Brugada, Blomstrom-Lundqvist, Deanfield and Janousek231], adenosine and magnesium sulphate [Reference Decker, McCormack and Cohen218].

6.5.1.6 Low cardiac output syndrome

Low cardiac output syndrome is multifactorial, related to CPB and circulatory arrest or to myocardial preservation, mechanical disruption of the myocardium, postinflammatory effects of bypass, and coronary manipulation, among others [Reference Wernovsky, Wypij, Jonas, Mayer, Hanley and Hickey129, Reference Vernon and Witte232]. LCOS may be more likely to happen when surgical intervention is delayed. It is critical to anticipate subtle changes that may indicate the inception of LCOS, including urine output, clinical perception of peripheral perfusion, and trends in heart rate and pressures, but this awareness may be related to the caregivers’ experience [Reference Tibby, Hatherill, Marsh and Murdoch233]. Persistent systemic hypotension in the setting of poor peripheral perfusion, elevated left atrial pressure, elevated serum lactate or decreased cerebral NIRS and other signs of left ventricular dysfunction should be promptly investigated. Any suspicion of coronary insufficiency should be addressed immediately, with possible re-exploration and revision. In the absence of coronary insufficiency, low cardiac output can be caused by primary myocardial dysfunction. Pharmacological support is therefore provided with vasoactive, inotropic and lusitropic drugs titrated to improve cardiac output with systemic afterload reduction and to enhance tissue perfusion markers.

6.5.1.7 Extracorporeal life support

In certain cases, mechanical circulatory support with ECLS or a ventricular assist device (VAD) may be indicated. In situations where adequate haemodynamics cannot be achieved or can be achieved only with high inotropic support (exposing the patient to significant myocardial oxygen consumption and progressive lactic acidosis and progressive alteration of tissue perfusion markers), a brief period of ECLS may help to bridge the patients to recovery. In such scenarios, it is vital to rule out residual defects that may need surgical revision prior to weaning the patient from the ECLS. The need for ECLS has been reported in around 20% of infants undergoing an ASO beyond the sixth week of life [Reference Bisoi, Ahmed, Malankar, Chauhan, Das and Sharma234].

6.5.1.8 Sedation and analgesia.

All medications should be given following the principle of minimal effective dosing. Analgesic and sedative strategies should encompass the provision of baseline comfort and the limitations of haemodynamic side effects. No combinations have proven superior, but it is important to remain consistent. Universal pain and sedation scores (i.e. the COMFORT scale) are required and will be used to evaluate needs and drug titration [Reference Ambuel, Hamlett, Marx and Blumer235]. Avoidance of prolonged use may help prevent subsequent withdrawal symptoms. Strategies to try to avoid withdrawal may include drug rotation, daily interruption of infusions and the use of long-acting agents; nonetheless, the most effective method is to avoid using opioids and benzodiazepines for more than 5 days [Reference Rawlinson and Howard236].

The most commonly prescribed combinations include opioids and benzodiazepines. Morphine and fentanyl are the most frequently used opioids for maintenance and breakthrough analgesia. Continuous infusions may be better tolerated and allow easier titration to minimal effective doses. It is important to keep in mind that the combination with nonopioid analgesia (i.e. paracetamol) reduces the requirements for opioids. Midazolam, the most commonly used agent for sedation, provides effective sedation and amnesia. Dexmedetomidine is a newer agent that has been approved for use in the European Union since 2011; it produces stable sedation without respiratory depression and decreases the need for other sedatives or analgesics [Reference Chrysostomou, Morell, Wearden, Sanchez-de-Toledo, Jooste and Beerman237Reference Tobias and Chrysostomou240]. The use of muscular relaxants should be avoided in the postoperative course of an ASO. It may occasionally be needed to promote ventilator synchrony with specific modalities. It may also be useful to induce hypothermia as part of the management of LCOS or after cardiac arrest, or in the event of pulmonary hypertension crisis or JET. Ventilated neonates on muscle relaxants have a higher than average overall mortality rate compared with neonates managed without muscle relaxants [Reference Martin, Bratton, Quint and Mayock241]. Neuromuscular blockade should be administered only in patients who are deeply sedated with appropriate monitoring [Reference Rawlinson and Howard236]. If necessary, its administration should be short-lived because it carries numerous inconveniences [Reference Tabarki, Coffinieres, Van Den Bergh, Huault, Landrieu and Sebire242]. Vecuronium is used most commonly; rocuronium and cisatracurium are also used frequently.

6.5.1.9 Ventilation and airway management.

Mechanical ventilation targets the maintenance of homeostasis (pH 7.35–7.45, PaCO2 35–45 mmHg, no hypoxaemia) while avoiding barotrauma. Ventilatory modalities and parameters need to be adapted to specific conditions such as pulmonary hypertension. Neonates with ongoing bleeding, unstable haemodynamics or rhythm abnormalities and those with delayed closure of the sternum should remain mechanically ventilated. All other patients should be weaned from mechanical ventilation over 12–24 h at the latest. Prevention of ventilator-associated pneumonia is essential in neonates who remain ventilated after the ASO. The main clinical bundles in the prevention recommendations are head-of-bed positioning, closed suction, oral care and patient repositioning [Reference Foglia, Meier and Elward243Reference Elward, Warren and Fraser246].

6.5.1.10 Fluid and electrolyte management.

Fluid overload can have a profound influence on the initial postoperative course; therefore a methodical follow-up of fluid intake and output is essential. IV fluids with 10% dextrose are given at 50% maintenance (2 ml/kg/day) for the first 24 h and then gradually used more liberally after that time. Hypotension due to hypovolaemia is treated with 5–10 ml/kg bolus infusions of 5% albumin, normal saline or FFP. Patients who are tenuous and anaemic ought to receive concentrated RBCs, and patients with persistent bleeding may require FFP, platelets and packed RBCs. On Day 2 postoperatively, fluids may be increased to 75% of requirements and then to 100% from Day 3 onwards.

6.5.1.11 Renal management.

Transient renal insufficiency and acute kidney injury are fairly common and associated with increased morbidity and mortality [Reference Gil-Ruiz Gil-Esparza, Alcaraz Romero, Romero Otero, Gil Villanueva, Sanavia Moran and Rodriguez Sanchez de la Blanca247Reference Watkins, Williamson, Davidson and Donahue251]. This disturbance is not exclusive to unstable patients and is multifactorial. A methodical evaluation of patients with suboptimal urine output is necessary and may require the estimation of a Paediatric Risk, Injury, Failure, Loss, End-Stage Renal Disease (pRIFLE) score. Furosemide is started 8–12 h after surgery (0.5–1 mg/kg boluses every 6–24 h or continuous infusion at 0.05–0.3 mg/kg/h).

6.5.1.12 Feeding and nutrition.

Enteral feeding is started when haemodynamics are stable. If enteral feeding cannot be started by Day 1 postoperatively, then total parenteral nutrition should be initiated. Mechanically ventilated patients can be fed via a nasogastric tube or a post-pyloric feeding tube if there is concern about gastro-oesophageal reflux. If the patient is breathing spontaneously and has stable respiratory mechanics, one can try oral feedings. In addition, ranitidine should be used prophylactically in the postoperative period.

ECG: electrocardiogram; ECLS: extracorporeal life support.

a Class of recommendation.

b Level of evidence.

c References.

Recommendations for intensive care postoperative management

7. Special topics

7.1 Atrial switch—is there a role for it?

7.1.1 Introduction

Over the years, atrial switch procedures have almost entirely yielded to the ASO in TGA IVS and are currently limited to rare indications. The long-term outcome following atrial switch procedures is reported to be 80% survival at 25–30 years [Reference Moons, Gewillig, Sluysmans, Verhaaren, Viart and Massin254Reference Wilson, Clarkson, Barratt-Boyes, Calder, Whitlock and Easthope258]. There is a significant late hazard function affecting this population, and sudden death occurs in 6–17% of patients [Reference Wilson, Clarkson, Barratt-Boyes, Calder, Whitlock and Easthope258]. Sudden death may relate to the occurrence of atrial arrhythmias [Reference Birnie, Tometzki, Curzio, Houston, Hood and Swan259] and loss of sinus rhythm [Reference Sun, Happonen, Bennhagen, Sairanen, Pesonen and Toivonen260]. Furthermore, the long-term outcome of these patients may entail a number of reoperations or interventional procedures in 10–20% of survivors, most commonly for baffle leak or obstruction [Reference Gewillig, Cullen, Mertens, Lesaffre and Deanfield261] or tricuspid regurgitation [Reference Duncan and Mee262].

7.1.2 Current role of atrial switches

Possible indications for atrial switch procedures in patients with TGA IVS are given in the ‘Recommendations for atrial switch’ (see section 7.1.4 ‘Treatment for late failure of the systemic right ventricle’ section).

7.1.3 Who still knows how—and is able—to perform this operation?

The currently limited application of the Senning and Mustard operations has deprived the younger generations of congenital heart surgeons of sufficient exposure to the many tips and pitfalls of the atrial switch procedures. Yet, mastering these techniques can be crucial for special circumstances. Exhaustive references dealing with the technical aspects of these procedures are available for both the Senning [Reference Barron, Jones and Brawn263Reference Senning265] and the Mustard [Reference Mustard266] operations, which are still indicated in conditions outside the scope of these guidelines.

7.1.4 Treatment for late failure of the systemic right ventricle

The treatment for late failure of the systemic right ventricle after the Senning or Mustard operation is controversial. The following two options are available:

  1. 1. Medical management and/or cardiac devices eventually followed by heart transplant, or

  2. 2. Conversion (staged) to anatomical repair.

7.1.4.1 Medical therapy for TGA patients with systemic right ventricular failure

Conservative management should include meticulous echocardiographic follow-up, with exercise testing when appropriate, to detect early changes in ventricular/valvular function and decrements in the patient’s functional status [Reference Duncan and Mee262]. Mild systemic right ventricular dysfunction with mild-to-moderate tricuspid regurgitation can be treated conservatively with afterload reduction and control of arrhythmias [Reference Duncan and Mee262, Reference Winter, Bouma, Groenink, Konings, Tijssen and van Veldhuisen267]. Afterload reduction, using angiotensin-converting enzyme inhibitors, β-blockers and diuretics, has proved useful in this setting [Reference Winter, Bouma, Groenink, Konings, Tijssen and van Veldhuisen267, Reference Hechter, Fredriksen, Liu, Veldtman, Merchant and Freeman268].

7.1.4.2 Cardiac devices for TGA patients with systemic right ventricular failure

Pacemaker implantation can restore physiological cardiac rhythm, with 11% of pacemaker dependency reported in the adult population of TGA patients [Reference Gelatt, Hamilton, McCrindle, Connelly, Davis and Harris269]. Electromechanical dyssynchrony, secondary to right bundle branch block or left ventricular pacing, may respond to cardiac resynchronization therapy [Reference Diller, Okonko, Uebing, Ho and Gatzoulis270, Reference Janousek, Tomek, Chaloupecky, Reich, Gebauer and Kautzner271]. Implantable cardioverter defibrillators may help prevent sudden death [Reference Triedman272]. The use of left VAD for RV failure following the Mustard operation has been described [Reference Kenleigh, Edens, Bates and Turek273].

7.1.4.3 Surgery for patients with TGA with right ventricular/tricuspid valve dysfunction

For cases with progressive right ventricular and tricuspid valve dysfunction, surgical therapy is indicated.

  1. 1. Tricuspid valve surgery: tricuspid valve repair or replacement may improve right ventricular function, but only if surgery is performed before significant right ventricular dysfunction ensues [Reference Warnes274]. It is unclear whether tricuspid valve replacement should be preferred to tricuspid valvuloplasty; however, surgical intervention is associated with a risk of early mortality (up to 10%) and the need for reoperation (in another 25%) [Reference van Son, Danielson, Huhta, Warnes, Edwards and Schaff275]. In general, tricuspid valve function and functional class improve significantly after surgery, and systemic right ventricular function is preserved [Reference Scherptong, Vliegen, Winter, Holman, Mulder and van der Wall276].

  2. 2. Pulmonary artery banding and conversion to anatomical repair: in the presence of reversible right ventricular dysfunction associated with well-maintained left ventricular and mitral valve function, conversion to anatomic correction may be considered.

However, with the exception of cases with long-standing pulmonary hypertension (e.g. for residual pulmonary venous obstruction) or ventricular outflow tract obstruction, the left ventricle must be retrained by incremental pulmonary artery banding [Reference Mavroudis and Backer277]. Gradual left ventricular training is vital because overzealous banding can induce left ventricular failure [Reference Poirier, Yu, Brizard and Mee278]. Interestingly, the increase in the left ventricular pressure may induce a rightward septal shift, with improved coaptation of the leaflets and consequent reduced regurgitation of the systemic atrioventricular valve. The result is reduced right ventricular volume load and improved function. Whether this approach can be considered a definitive palliation is not yet clear [Reference Duncan and Mee262, Reference Warnes274, Reference van Son, Danielson, Huhta, Warnes, Edwards and Schaff275, Reference Mavroudis and Backer277]. Nonetheless, it can act as a bridge to transplant [Reference Poirier, Yu, Brizard and Mee278].

7.1.4.4 Methods for conversion of atrial switch to an ASO

Left ventricular training in children or older patients is a much slower process than it is in infants and is achievable by different strategies of incremental pulmonary artery banding:

  1. 1. Single, long-standing (≥1 year) pulmonary artery banding, in which the patient gradually outgrows an initially loose band [Reference Poirier, Yu, Brizard and Mee278].

  2. 2. Multiple surgical procedures (2–3 stages) with progressive tightening of the band [Reference Poirier, Yu, Brizard and Mee278].

  3. 3. Application of a telemetric adjustable pulmonary artery band, undertaking a variety of cardiac fitness protocols [Reference Corno279, Reference Dibardino, Kleeman and Bove280].

7.1.4.5 Indications and timing for conversion of atrial switch to an ASO

There is an age-dependent time limit for preparatory pulmonary artery banding to recondition the left ventricle [Reference Di Donato, Fujii, Jonas and Castaneda102, Reference Helvind, McCarthy, Imamura, Prieto, Sarris and Drummond-Webb122, Reference Warnes274]. Clinically, the response to left ventricular training appears inconsistent among adolescents and adults [Reference Mavroudis and Backer277, Reference Poirier, Yu, Brizard and Mee278, Reference Padalino, Stellin, Brawn, Fasoli, Daliento and Milanesi281]. This response is a possible consequence of inadequate myocardial perfusion in the presence of suddenly increased cardiac work during pulmonary banding and induced myocardial hypertrophy [Reference Di Donato, Fujii, Jonas and Castaneda102, Reference Poirier, Yu, Brizard and Mee278]. In older patients, failure to achieve adequate reconditioning of the left ventricle increases the risk of death and calls for timely transplant [Reference Helvind, McCarthy, Imamura, Prieto, Sarris and Drummond-Webb122]. In general, however, strict contraindications for left ventricular reconditioning and/or ultimate anatomical correction do not exist [Reference Padalino, Stellin, Brawn, Fasoli, Daliento and Milanesi281].

7.1.4.6 Pulmonary artery banding—operative and postoperative management

The band is gradually tightened until a drop in the systemic blood pressure and a rise in the CVP are observed, together with a decrease in oxygen saturation or the development of rhythm disturbances; the band is then loosened slightly [Reference Duncan and Mee262, Reference Daebritz, Tiete, Sachweh, Engelhardt, von Bernuth and Messmer282]. This step usually corresponds to an increment in left ventricular pressure of 20–50 mmHg. TOE can identify any shift of the ventricular septum and reduction of tricuspid regurgitation and right ventricular dysfunction. Conversely, if TOE demonstrates any deterioration in left ventricular function or the manifestation of mitral regurgitation, the band should be loosened immediately [Reference Duncan and Mee262].

7.1.4.7 Interim follow-up during left ventricular training

Left ventricular function and pressures are periodically reassessed (e.g. every 3 months) using echocardiography, magnetic resonance imaging (MRI) and cardiac catheterization. Criteria for left ventricular preparedness include ventricular ejection fraction of ≥55%, less-than-mild mitral regurgitation, ventricular systolic pressure ≥80% of systemic blood pressure, ventricular end-diastolic pressure ≤10 mmHg, normal indexed ventricular mass (≥60 g/m2) and normal wall thickness [Reference Duncan and Mee262, Reference Poirier, Yu, Brizard and Mee278]. If these criteria are not met, pulmonary artery banding is repeated. Roughly one to two band-tightening procedures over a 1-year period may be needed [Reference Duncan and Mee262, Reference Poirier, Yu, Brizard and Mee278, Reference Benzaquen, Webb, Colman and Therrien283]. If left ventricular dysfunction is observed or if atrial arrhythmias progress, the banding is taken down.

Patients are listed for transplant when their condition deteriorates to end-stage heart failure [Reference Poirier, Yu, Brizard and Mee278].

7.1.4.8 Anatomical correction—operative and postoperative management

Along with take-down of atrial repair and conversion to anatomical correction [Reference Mee284], additional procedures may be needed to manage neoaortic insufficiency or atrial arrhythmias [Reference Mavroudis and Backer277]. The immediate postoperative therapy must comprise aggressive reduction of both pre- and afterload (using phenoxybenzamine and nitroprusside) and adequate inotropic support (using dopamine, dobutamine and milrinone). Nitroglycerine therapy may help to prevent coronary vasospasm and to control pulmonary artery vasoreactivity. Fluid challenges are contraindicated. Patients stay paralyzed and ventilated for at least 24 h, and chronic afterload reduction medication is started orally once the patient is extubated [Reference Mavroudis and Backer277].

7.1.4.9 Results for anatomic conversion of TGA after the mustard or senning operation.

The median duration of pulmonary artery banding for left ventricular training is 13 months (maximum duration up to 5 years). Approximately one-half of these patients survive after an ASO in New York Heart Association functional class I or II, whereas another one-quarter need a heart transplant [Reference Duncan and Mee262, Reference Mavroudis and Backer277, Reference Daebritz, Tiete, Sachweh, Engelhardt, von Bernuth and Messmer282, Reference Chang, Wernovsky, Wessel, Freed, Parness and Perry285, Reference van Son, Reddy, Silverman and Hanley286]. Additional morbidity occurs in these patients due to progressive aortic insufficiency and arrhythmias [Reference Mavroudis and Backer277, Reference Poirier, Yu, Brizard and Mee278].

7.1.4.10 Heart transplant.

Patients presenting with advanced right ventricular or biventricular failure, severe tricuspid or mitral dysfunction, pulmonary valve abnormalities precluding its use as a neoaortic valve, resilient arrhythmias or heart block, especially beyond adolescence, should not be offered left ventricle training measures with the ultimate intention of anatomical correction and should be enrolled in the heart transplant pathway [Reference Duncan and Mee262]. Nowadays, heart transplant is a well-established treatment strategy [Reference Hetzer, Weng and Delmo Walter287] and is likely to be a superior option to anatomical conversion from a functional aspect. However, it is challenging for both patient and clinician and, even more importantly, has limited application due to the shortage of donor organs [Reference Scherptong, Vliegen, Winter, Holman, Mulder and van der Wall276, Reference Canter, Shaddy, Bernstein, Hsu, Chrisant and Kirklin288]. Therefore, currently, the most reasonable approach for patients with late right ventricular failure after an atrial switch repair is probably to integrate left ventricular reconditioning and an anatomical correction protocol into a cardiac transplant programme that can serve as a bailout solution [Reference Poirier, Yu, Brizard and Mee278].

ASO: arterial switch operation; IVS: intact ventricular septum; TGA: transposition of the great arteries.

a Class of recommendation.

b Level of evidence.

c References.

Recommendations for atrial switch

a Class of recommendation.

b Level of evidence.

c References.

Recommendations for late left ventricular training

7.2 Cardiology follow-up protocols

Recommendations for follow-up of ASO patients are challenged by several factors: (i) current consensus on appropriate time interval and modality of surveillance imaging is still lacking; (ii) symptoms are rare and can be atypical, particularly in cases of coronary obstruction; (iii) a clear treatment strategy for sub-clinical anatomical or functional findings is not available; and (iv) during long-term follow-up, the effects of superimposed coronary artery disease on the operated coronary arteries are still unknown [Reference Villafane, Lantin-Hermoso, Bhatt, Tweddell, Geva and Nathan148].

Nevertheless, according to the common standard of care, short-term follow-up during the first year after an ASO should include clinical visits at 1, 3, 6 and12 months postoperatively. During mid- and long-term follow-up, children should be examined yearly and adults, every second year [Reference Wernovsky, Rome, Tabbutt, Rychik, Cohen and Paridon292].

Each visit should include history taking, clinical examination, 12-lead ECG and transthoracic echocardiography. A 24-h ECG should be performed annually in small children, because it may indirectly detect myocardial ischaemia. Regular exercise testing should be used to screen for myocardial ischaemia as soon as the patient is old enough to cooperate.

Well-known sequelae after an ASO include obstruction of the coronary arteries with related myocardial ischaemia, left ventricular dysfunction, stenoses of the pulmonary artery side branches, RVOT obstruction, neoaortic valve insufficiency and dilatation of the aortic root [Reference Tobler, Williams, Jegatheeswaran, Van Arsdell, McCrindle and Greutmann145, Reference Angeli, Raisky, Bonnet, Sidi and Vouhé293]. Therefore, follow-up should be targeted to detect such lesions.

Supravalvular pulmonary stenosis, particularly in the side branches of the pulmonary arteries, occurs mainly early after an ASO. Transthoracic echocardiography is the modality of choice for detecting RVOT stenosis. Additional imaging is frequently required, typically cardiac MRI, which has the advantages of providing clear anatomical delineation of the pulmonary arteries and additional functional information; therefore, cardiac MRI is considered the modality of choice for determining the need for a catheter-guided intervention involving the pulmonary arteries [Reference Valsangiacomo Buechel, Grosse-Wortmann, Fratz, Eichhorn, Sarikouch and Greil294, Reference Cohen, Eidem, Cetta, Fogel, Frommelt and Ganame295].

Neoaortic root dilatation and neoaortic valve regurgitation are the most frequently observed postoperative sequelae during long-term follow-up. Both findings can be detected and quantified using transthoracic echocardiography. However, exact quantification of the diameter of the aortic root and of regurgitant flow through the aortic valve, as well as of size and function of the left ventricle, may require examination by cardiac MRI [Reference Fratz, Chung, Greil, Samyn, Taylor and Valsangiacomo Buechel296].

Obstructed coronary arteries occur in 5–7% of survivors and remain the most common cause of morbidity and mortality after the ASO procedure [Reference Legendre, Losay, Touchot-Kone, Serraf, Belli and Piot297, Reference Ou, Khraiche, Celermajer, Agnoletti, Le Quan Sang and Thalabard298]. Invasive X-ray coronary angiography is still considered the gold standard for evaluation of coronary patency or obstruction; however, modern ECG-triggered computed tomography (CT) angiography provides excellent spatial resolution, has been validated in large studies [Reference Cho, Chang, Shin, Sung and Lin299] and can be used for morphological assessment in young adults after an ASO [Reference Ou, Celermajer, Marini, Agnoletti, Vouhé and Brunelle300].

In children, the use of coronary CT angiography is limited by the burden of radiation and the fast heart rate. Cardiac MRI is not validated for final evaluation of the coronary lumen but provides crucial functional information about myocardial perfusion (from first-pass perfusion images) and about the presence of myocardial scars (from late-enhancement images). In patients undergoing an ASO, cardiac MRI first pass has been shown to have slightly better diagnostic performance than positron emission tomography scans [Reference Tobler, Motwani, Wald, Roche, Verocai and Iwanochko301]. This technique has also been validated in children [Reference Buechel, Balmer, Bauersfeld, Kellenberger and Schwitter302, Reference Taylor, Dymarkowski, Hamaekers, Razavi, Gewillig and Mertens303]. Thus, the current recommendation is to perform noninvasive screening for myocardial ischaemia, with exercise testing (in older children and adults) and 24-h ECG (in younger children), in combination with cardiac MRI.

7.3 Arrhythmias

Postoperative early and late arrhythmias may have various causes but can also be related to subacute or acute myocardial ischaemia due to impaired coronary artery blood flow. Patients with certain unusual coronary patterns (including those with an intramural or single coronary artery) have a significantly increased mortality risk [Reference Prêtre, Tamisier, Bonhoeffer, Mauriat, Pouard and Sidi139, Reference Pasquali, Hasselblad, Li, Kong and Sanders143, Reference Planche, Bruniaux, Lacour-Gayet, Kachaner, Binet and Sidi154, Reference Day, Laks and Drinkwater304, Reference Yamaguchi, Hosokawa, Imai, Kurosawa, Yasui and Yagihara305]. Arrhythmia problems encountered include:

  1. 1. Supraventricular tachycardia and sinus node dysfunction are rarely seen after an ASO but are common after senning or mustard procedures [Reference el-Said, Rosenberg, Mullins, Hallman, Cooley and McNamara306Reference Martin, Smith, Hernandez and Weldon308].

  2. 2. Sudden death: of all forms of congenital cardiac lesions, in the adult population, TGA is associated with one of the highest risks of sudden cardiac death [Reference Gelatt, Hamilton, McCrindle, Connelly, Davis and Harris269]. For children, adverse prognostic indicators are right ventricular dysfunction and QRS prolongation [Reference Wong, Finucane, Kerr, O’donnell, West and Gentles146, Reference Hayashi, Kurosaki, Echigo, Kado, Fukushima and Yokota309, Reference Yamazaki, Yamamoto, Sakamoto, Ishihara, Iwata and Matsumura310].

Thus, unlike in TGA patients with atrial switch repairs, arrhythmias are uncommon after an ASO (≤5%), possibly due to limited atrial manipulation [Reference Rhodes, Wernovsky, Keane, Mayer, Shuren and Dindy311]. Most patients who have had the ASO have, in fact, preserved sinus node function at long-term follow-up [Reference Rhodes, Wernovsky, Keane, Mayer, Shuren and Dindy311]. However, ASO patients are not completely free from arrhythmic complications. Longer aortic cross-clamp time and early age at time of operation have been identified as risk factors for rhythm disturbances among paediatric cardiac patients [Reference Delaney, Moltedo, Dziura, Kopf and Snyder312].

ASO: arterial switch operation; CMR: cardiovascular magnetic resonance; ECG: electrocardiogram; TGA: transposition of the great arteries.

a Class of recommendation.

b Level of evidence.

c References.

Recommendations for cardiology follow-up and arrhythmias

8. Reoperations and Interventions for Management of Late Complications

8.1 Introduction

With the transition from atrial to arterial repair of TGA, complications requiring reoperation have shifted mainly from the inflow to the outflow of the ventricles [Reference Losay, Touchot, Serraf, Litvinova, Lambert and Piot313, Reference Horer, Schreiber, Cleuziou, Vogt, Prodan and Busch314]. Most reoperations are performed during the first year after an ASO. This initial hazard phase is followed by a period of very low risk for reoperation, with a slightly ascending late hazard phase due to the increasing need for pulmonary artery and neoaortic valve surgery [Reference Losay, Touchot, Serraf, Litvinova, Lambert and Piot313]. Most (>80%) late reoperations are performed within the first 10 years after an ASO. Survival and functional outcome seem unaffected by the need for reintervention [Reference Angeli, Raisky, Bonnet, Sidi and Vouhé293]. Freedom from reoperation after a neonatal ASO is about 80% at 20 years [Reference Fricke, D’Udekem, Richardson, Thuys, Dronavalli and Ramsay3, Reference Lim, Kim, Lee and Kim5, Reference Oda, Nakano, Sugiura, Fusazaki, Ishikawa and Kado6, Reference Khairy, Clair, Fernandes, Blume, Powell and Newburger141, Reference Raju, Burkhart, Durham, Eidem, Phillips and Li169, Reference Lalezari, Bruggemans, Blom and Hazekamp171, Reference Horer, Schreiber, Cleuziou, Vogt, Prodan and Busch314]. The overall catheter reintervention rate is approximately 18% at 10 years and 25% at 25 years post-ASO [Reference Khairy, Clair, Fernandes, Blume, Powell and Newburger141, Reference Rodriguez Puras, Cabeza-Letran, Romero-Vazquianez, Santos de Soto, Hosseinpour and Gil Fournier315]. Most reinterventions are carried out during childhood [Reference Kempny, Wustmann, Borgia, Dimopoulos, Uebing and Li4, Reference Khairy, Clair, Fernandes, Blume, Powell and Newburger141, Reference Tobler, Williams, Jegatheeswaran, Van Arsdell, McCrindle and Greutmann145]. The risk for reintervention in adulthood is significantly higher for those having already had one reintervention during childhood [Reference Tobler, Williams, Jegatheeswaran, Van Arsdell, McCrindle and Greutmann145].

8.2 Reoperations for neopulmonary outflow tract lesions

8.2.1 Relief of neopulmonary outflow tract obstruction

8.2.1.1 Neopulmonary outflow tract obstruction: prevalence and incidence

Neopulmonary outflow tract obstruction is the most frequent cause for reoperation [Reference Haas, Wottke, Poppert and Meisner316Reference Nogi, McCrindle, Boutin, Williams, Freedom and Benson318]. Obstruction may occur at multiple levels following an ASO but most frequently involves the pulmonary arteries, with a reported incidence of 1–42% [Reference Prêtre, Tamisier, Bonhoeffer, Mauriat, Pouard and Sidi139, Reference Ullmann, Gorenflo, Bolenz, Sebening, Goetze and Arnold166, Reference Hutter, Kreb, Mantel, Hitchcock, Meijboom and Bennink167, Reference Rudra, Mavroudis, Backer, Kaushal, Russell and Stewart173, Reference Angeli, Raisky, Bonnet, Sidi and Vouhé293]. Neopulmonary valve stenosis tends to develop primarily during the first year after repair [Reference Wernovsky, Mayer, Jonas, Hanley, Blackstone and Kirklin319], but there is a persistent constant low risk thereafter [Reference Losay, Touchot, Serraf, Litvinova, Lambert and Piot313, Reference Wernovsky, Mayer, Jonas, Hanley, Blackstone and Kirklin319].

8.2.1.2 Neopulmonary outflow tract obstruction: patient-related risk factors

  1. 1. Younger age at operation and lower birth weight [Reference Wernovsky, Mayer, Jonas, Hanley, Blackstone and Kirklin319Reference Vargo, Mavroudis, Stewart and Backer321].

  2. 2. Coronary artery pattern (e.g. left coronary artery arising from the right-facing sinus) [Reference Wernovsky, Mayer, Jonas, Hanley, Blackstone and Kirklin319Reference Vargo, Mavroudis, Stewart and Backer321].

  3. 3. Size mismatch between the great arteries and a smaller native aortic annulus [Reference Bove, De Meulder, Vandenplas, De Groote, Panzer and Suys322].

  4. 4. Side-by-side relationship of the great arteries [Reference Williams, Quaegebeur, Kirklin and Blackstone320].

  5. 5. Coexisting aortic coarctation (with a small native aortic annulus) [Reference Williams, Quaegebeur, Kirklin and Blackstone320].

  6. 6. Rapid somatic growth [Reference Angeli, Raisky, Bonnet, Sidi and Vouhé293, Reference Prifti, Crucean, Bonacchi, Bernabei, Murzi and Luisi323].

  7. 7. Remnant ductal tissue causing left pulmonary artery stenosis [Reference Lupinetti, Bove, Minich, Snider, Callow and Meliones324, Reference Paillole, Sidi, Kachaner, Planche, Belot and Villain325].

  8. 8. Earlier institutional experience [Reference Williams, Quaegebeur, Kirklin and Blackstone320].

8.2.1.3 Neopulmonary outflow tract obstruction: surgery-related risk factors

  1. 1. Diffuse hypoplasia of the main pulmonary artery may be secondary to several mechanisms:

  1. 1. Inadequate mobilization of the branch pulmonary arteries [Reference Wernovsky, Hougen, Walsh, Sholler, Colan and Sanders326, Reference Spiegelenberg, Hutter, van de Wal, Hitchcock, Meijboom and Harinck327].

  2. 2. Technique used for reconstruction of the proximal pulmonary artery [Reference Lupinetti, Bove, Minich, Snider, Callow and Meliones324, Reference Paillole, Sidi, Kachaner, Planche, Belot and Villain325, Reference Zeevi, Keane, Perry and Lock328]. Distortion and stretching of the main and branch pulmonary arteries occur as a result of the Lecompte manoeuvre [Reference Kuroczynski, Kampmann, Choi, Hilker, Wippermann and David329].

  3. 3. Posterior compression of the neopulmonary valve and main pulmonary artery by the neoaorta, also as a result of the Lecompte manoeuvre [Reference Massin, Nitsch, Dabritz, Seghaye, Messmer and von Bernuth330].

  4. 4. Pulmonary artery banding causing supravalvular stenosis [Reference Nakanishi, Momoi, Satoh, Yamada, Terada and Nakazawa331, Reference Serraf, Bruniaux, Lacour-Gayet, Sidi, Kachaner and Bouchart332].

  5. 5. Growth failure of the valve annulus [Reference Nogi, McCrindle, Boutin, Williams, Freedom and Benson318, Reference Nakanishi, Momoi, Satoh, Yamada, Terada and Nakazawa331].

  6. 6. Use of prosthetic material in reconstruction of the neopulmonary sinuses [Reference Williams, Quaegebeur, Kirklin and Blackstone320].

  1. 2. Discrete, circumferential narrowing at the suture line, leading to inadequate growth of the pulmonary anastomotic site resulting in a more discrete type of stenosis [Reference Angeli, Raisky, Bonnet, Sidi and Vouhé293, Reference Wernovsky, Mayer, Jonas, Hanley, Blackstone and Kirklin319].

8.2.1.4 Neopulmonary outflow tract obstruction: indications for treatment.

Reoperation or intervention is indicated when a gradient >50 mmHg at any level in the neopulmonary outflow tract is detected on routine echo Doppler evaluation [Reference Angeli, Raisky, Bonnet, Sidi and Vouhé293]. Supravalvular pulmonary stenosis should be relieved early because it may be associated with growth failure of the valve annulus [Reference Raja, Shauq and Kaarne333, Reference Santoro, Di Carlo, Formigari and Ballerini334] and may induce asymmetrical distribution of pulmonary flow [Reference Massin, Nitsch, Dabritz, Seghaye, Messmer and von Bernuth330].

8.2.1.5 Neopulmonary outflow tract obstruction: intervention.

Some patients who have had an ASO may need a reintervention to alleviate pulmonary branch artery stenosis. Although the arterial diameter could be increased in some cases using balloon dilation, the improvements did not last, and a high recurrence rate was observed. These unfavourable results led to the use of stents, which were significantly more effective [Reference Formigari, Santoro, Guccione, Giamberti, Pasquini and Grigioni335]. The risks of early dissection and vessel rupture are known and manageable. More recently, a potentially more serious complication has been described—creation of an aortopulmonary window. This complication cannot be avoided entirely because overdilation is necessary to achieve long-term success, but cardiologists should be aware of it [Reference Tzifa, Papagiannis and Qureshi336, Reference Torres, Sanders, Vincent, El-Said, Leahy and Padera337].

RVOT: right ventricular outflow tract.

a Class of recommendation.

b Level of evidence.

c References.

Recommendations for late neopulmonary outflow tract obstruction

8.2.2 Treatment of neopulmonary valve regurgitation
8.2.2.1 Neopulmonary valve regurgitation: prevalence and incidence.

Neopulmonary valve regurgitation occurs after an ASO with an incidence varying from 9% to 80% [Reference Gleason, Chin, Andrews, Barber, Helton and Murphy338, Reference Martin, Snider, Bove, Serwer, Rosenthal and Peters339]. At least moderate neopulmonary valve regurgitation was present in 6.6% of the cases in the series of Khairy et al. [Reference Khairy, Clair, Fernandes, Blume, Powell and Newburger141].

8.2.2.2 Neopulmonary valve regurgitation: potential risk factors

  1. 1. Valve distortion secondary to anterior displacement of the neopulmonary arterial root by a redundant posterior neoaorta.

  2. 2. Valve misalignment during patch reconstruction of the neopulmonary artery root.

  3. 3. Loss of the sinotubular junction of the neopulmonary artery root following single-patch reconstruction of the coronary defects.

  4. 4. Partial commissural detachment to harvest a paracommissural or intramural coronary artery.

8.2.2.3 Neopulmonary valve regurgitation: indications for treatment

Reported cases of treated neopulmonary valve regurgitation are sporadic and usually embedded in large series of late results from ASOs [Reference Khairy, Clair, Fernandes, Blume, Powell and Newburger141, Reference Hovels-Gurich, Seghaye, Ma, Miskova, Minkenberg and Messmer340]. An increasing number of such cases are anticipated as the ASO population gets older. Indications for neopulmonary valve surgery probably replicate those for any long-standing pulmonary-valve regurgitation affecting right ventricular function [Reference Geva341]. Surgical pulmonary valvuloplasty is attempted with increasing frequency [Reference Khairy, Clair, Fernandes, Blume, Powell and Newburger141]. However, pulmonary valve replacement still is often required with a preference for biological prostheses to allow later transcatheter implantation of other prostheses [Reference Eicken, Ewert, Hager, Peters, Fratz and Kuehne342, Reference Thanopoulos, Giannakoulas and Arampatzis343], although some concern has been raised about potential coronary compression [Reference Biermann, Schonebeck, Rebel, Weil and Dodge-Khatami344].

a Class of recommendation.

b Level of evidence.

c References.

Recommendations for reoperations for late neopulmonary valve regurgitation

8.3 Reoperations for left ventricular outflow tract lesions

8.3.1 Treatment of neoaortic valve regurgitation

8.3.1.1 Neoaortic regurgitation and root dilatation: prevalence and incidence

After an ASO, the native pulmonary valve faces the high-pressure systemic circulation. It is still unclear whether it can maintain long-term competence [Reference Angeli, Raisky, Bonnet, Sidi and Vouhé293, Reference Vargo, Mavroudis, Stewart and Backer321]. Neoaortic regurgitation is the second most common indication for reoperation after an ASO [Reference Raju, Burkhart, Durham, Eidem, Phillips and Li169, Reference Haas, Wottke, Poppert and Meisner316], needed in 5–22% of cases [Reference Delmo Walter, Huebler, Alexi-Meshkishvili, Sill, Berger and Hetzer345Reference Schwartz, Gauvreau, del Nido, Mayer and Colan348]. It was more frequent in the early years of ASOs, occurring in up to 55% of patients undergoing a two-stage operation [Reference Martin, Ettedgui, Qureshi, Gibbs, Baker and Radley-Smith349, Reference Lange, Cleuziou, Horer, Holper, Vogt and Tassani-Prell350]. In the current era of neonatal ASOs, moderate-to-severe neoaortic regurgitation is less frequent (<7%) [Reference Marino, Wernovsky, McElhinney, Jawad, Kreb and Mantel346Reference Schwartz, Gauvreau, del Nido, Mayer and Colan348]; the need for reoperation at 15 years is ≤3% [Reference Haas, Wottke, Poppert and Meisner316]. Of concern, however, is the high prevalence of trivial-to-mild neoaortic regurgitation and neoaortic root dilation, with some evidence of progression [Reference Haas, Wottke, Poppert and Meisner316, Reference Marino, Wernovsky, McElhinney, Jawad, Kreb and Mantel346, Reference Lange, Cleuziou, Horer, Holper, Vogt and Tassani-Prell350Reference Hwang, Kim, Kwak, Lee, Kim and Rho352]. Aortic root dilation usually stabilizes over time [Reference Schwartz, Gauvreau, del Nido, Mayer and Colan348, Reference Hutter, Thomeer, Jansen, Hitchcock, Faber and Meijboom353]. Fortunately, neoaortic valve intervention is associated with good outcomes [Reference Mavroudis, Stewart, Backer, Rudra, Vargo and Jacobs354, Reference Losay, Touchot, Capderou, Piot, Belli and Planche355].

8.3.1.2 Neoaortic regurgitation and root dilatation: patient-related risk factors

  1. 1. Intrinsic weakness of native pulmonary artery and valve tissue in facing systemic pressure [Reference Raju, Burkhart, Durham, Eidem, Phillips and Li169, Reference del Nido and Schwartz356Reference Niwa, Perloff, Bhuta, Laks, Laks, Drinkwater and Child359], although others have refuted this hypothesis [Reference Bottio, Thiene, Tarzia, Rizzoli and Gerosa360].

  2. 2. Previous pulmonary banding, distorting the native pulmonary artery root [Reference Vargo, Mavroudis, Stewart and Backer321, Reference Marino, Wernovsky, McElhinney, Jawad, Kreb and Mantel346, Reference Losay, Touchot, Capderou, Piot, Belli and Planche355].

  3. 3. The diameter of the neoaortic root appears positively related with mild-to-moderate neoaortic regurgitation [Reference Bove, De Meulder, Vandenplas, De Groote, Panzer and Suys322, Reference Co-Vu, Ginde, Bartz, Frommelt, Tweddell and Earing361, Reference Lalezari, Mahtab, Bartelings, Wisse, Hazekamp and Gittenberger-de Groot362]. Freedom from neoaortic root dilation at 1, 5, 10 and 15 years after an ASO is 84%, 67%, 47% and 32%, respectively [Reference Co-Vu, Ginde, Bartz, Frommelt, Tweddell and Earing361]. Others, however, observed that rapid dilation of the neoaorta is limited to the first year of life [Reference Hutter, Thomeer, Jansen, Hitchcock, Faber and Meijboom353].

  4. 4. Non-facing commissures [Reference Michalak, Moll, Moll, Dryzek, Moszura and Kopala363].

  5. 5. Abnormal coronary artery anatomy [Reference Schwartz, Gauvreau, del Nido, Mayer and Colan348, Reference Formigari, Toscano, Giardini, Gargiulo, Di Donato and Picchio364].

  6. 6. Preoperative pulmonary valve regurgitation [Reference Hwang, Kim, Kwak, Lee, Kim and Rho352].

  7. 7. Acute angulation of the aortic arch following posterior translocation of the ascending aorta [Reference Agnoletti, Ou, Celermajer, Boudjemline, Marini and Bonnet365].

  8. 8. Impaired growth of the neoaorta [Reference Formigari, Toscano, Giardini, Gargiulo, Di Donato and Picchio364].

8.3.1.3 Neoaortic regurgitation and root dilatation: surgery-related risk factors

  1. 1. The trap-door technique may cause distortion of the geometry of the sinotubular junction [Reference Formigari, Toscano, Giardini, Gargiulo, Di Donato and Picchio364], although others have disputed this suggestion [Reference Jhang, Shin, Park, Yun, Kim and Ko366]. Very large buttons for translocation of the coronary arteries can also distort.

  2. 2. Aortic transection with disruption of vasa vasorum may alter the structure of the neoaorta [Reference McMahon, Ravekes, Smith, Denfield, Pignatelli and Altman347, Reference Murakami, Nakazawa, Momma and Imai367].

8.3.1.4 Neoaortic root dilation and neoaortic valve regurgitation: treatment

Surgery on the neoaortic root or valve is rare after an ASO and is needed mainly in children aged >1 year at the time of the ASO, particularly those undergoing conversion of an atrial switch [Reference Fricke, Brizard, D’Udekem and Konstantinov368]. The indications for aortic valve surgery are the same as those for adults with aortic regurgitation [Reference Marino, Bridges and Paridon369]. The indications and timing for repair of neoaortic root dilation are unclear [Reference Co-Vu, Ginde, Bartz, Frommelt, Tweddell and Earing361]. Nevertheless, elective neoaortic root operations after an ASO have been reported for patients with neoaortic root z-scores of ≥3 [Reference Schwartz, Gauvreau, del Nido, Mayer and Colan348]. If neoaortic regurgitation occurs early, the patient should be closely monitored [Reference Vargo, Mavroudis, Stewart and Backer321]. Importantly, the operation should be advised before the development of severe aortic regurgitation [Reference Raju, Burkhart, Durham, Eidem, Phillips and Li169]. Surgical options adopted for neoaortic valve regurgitation with or without neoaortic root dilation include:

  1. 1. Valve-sparing aortic root repair, preventing the need for anticoagulation [Reference Mavroudis, Stewart, Backer, Rudra, Vargo and Jacobs354, Reference Imamura, Drummond-Webb, McCarthy and Mee370].

  2. 2. Prosthetic valve replacement [Reference Oda, Nakano, Sugiura, Fusazaki, Ishikawa and Kado6, Reference Mavroudis, Stewart, Backer, Rudra, Vargo and Jacobs354, Reference Losay, Touchot, Capderou, Piot, Belli and Planche355, Reference Lalezari, Mahtab, Bartelings, Wisse, Hazekamp and Gittenberger-de Groot362, Reference Fricke, Brizard, D’Udekem and Konstantinov368, Reference Alexi-Meskishvili, Photiadis and Nurnberg371].

  3. 3. Bentall operation [Reference Oda, Nakano, Sugiura, Fusazaki, Ishikawa and Kado6, Reference Fricke, Brizard, D’Udekem and Konstantinov368].

  4. 4. Switch-back operation (Ross after an ASO) [Reference Hazekamp, Schoof, Suys, Hutter, Meijboom and Ottenkamp372, Reference Vicente, Ferreira, Klamt, Manso, Filho and Carlotti373].

a Class of recommendation.

b Level of evidence.

c References.

Recommendations for reoperations for late neoaortic valve regurgitation

8.3.2 Relief of neoaortic outflow tract obstruction

Left-sided obstruction needing reoperation after an ASO is considerably less frequent than neopulmonary outflow tract obstruction, with freedom from reinterventions of up to 99% at 10 years after an ASO [Reference Williams, Quaegebeur, Kirklin and Blackstone320, Reference Hourihan, Colan, Wernovsky, Maheswari, Mayer and Sanders374, Reference Leobon, Belli, Ly, Kortas, Le Bret and Sigal-Cinqualbre375]. However, at least mild late neoaortic stenosis was found in 3.2% of ASO patients [Reference Khairy, Clair, Fernandes, Blume, Powell and Newburger141]. Reintervention for left-sided obstruction after an ASO may consist of direct relief of the residual LVOT obstruction or of patch angioplasty of the ascending aorta [Reference Williams, Quaegebeur, Kirklin and Blackstone320]. Neoaortic valvular stenosis with a small annulus after an ASO can be managed using the Konno procedure [Reference Leobon, Belli, Ly, Kortas, Le Bret and Sigal-Cinqualbre375, Reference Kosaka, Kurosawa and Nagatsu376].

8.4 Reoperations for coronary lesions

Coronary obstructions may develop even after a successful ASO and can lead to coronary events [Reference Tamisier, Ouaknine, Pouard, Mauriat, Lefebvre and Sidi377]. Coronary events show a bimodal pattern with a rapidly declining early phase and a slowly increasing late phase [Reference Legendre, Losay, Touchot-Kone, Serraf, Belli and Piot297]. The left main coronary artery is more frequently affected [Reference Oda, Nakano, Sugiura, Fusazaki, Ishikawa and Kado6, Reference Ou, Khraiche, Celermajer, Agnoletti, Le Quan Sang and Thalabard298].

8.4.1 Mechanisms for long-term declining coronary function

Progressive proximal eccentric intimal thickening occurs in up to 89% of the coronary arteries after an ASO [Reference Pedra, Pedra, Abizaid, Braga, Staico and Arrieta378]. The complexity of coronary patterns and the modality of coronary transfer could prompt flow abnormalities, leading to increased shear stress and progressive fibrocellular intimal thickening [Reference Angeli, Raisky, Bonnet, Sidi and Vouhé293, Reference Legendre, Losay, Touchot-Kone, Serraf, Belli and Piot297, Reference Pedra, Pedra, Abizaid, Braga, Staico and Arrieta378, Reference Bartoloni, Bianca, Patane and Mignosa379]. Other suggested causes of progressive coronary occlusion include ostial fibrosis at the suture line, kinking or stretching with reactive injury from surgical manipulation [Reference Vargo, Mavroudis, Stewart and Backer321, Reference Bonnet, Bonhoeffer, Piechaud, Aggoun, Sidi and Planche380, Reference Tanel, Wernovsky, Landzberg, Perry and Burke381]. A hypothetical compensatory mechanism is the greater capacity of collateralization, neovascularization and cellular proliferation of infants compared with adults [Reference Tanel, Wernovsky, Landzberg, Perry and Burke381]. In reality, however, infant coronary collateralization is unpredictable, and survival relies mostly on the number of arterial segments remaining patent [Reference Mavroudis, Stewart, Backer, Rudra, Vargo and Jacobs354]. As the ASO population approaches adulthood, superimposed variations in coronary artery anatomy will conceivably increase the risk factors for atherosclerotic disease and ischaemic events [Reference Vargo, Mavroudis, Stewart and Backer321]. Therefore, these patients should undergo life-long close monitoring of myocardial perfusion [Reference Angeli, Formigari, Pace Napoleone, Oppido, Ragni and Picchio382].

8.4.2 Coronary lesions: prevalence, incidence and diagnosis

The known incidence of late coronary stenosis requiring reintervention varies from <3% to 10% [Reference Khairy, Clair, Fernandes, Blume, Powell and Newburger141, Reference Pasquali, Hasselblad, Li, Kong and Sanders143, Reference Legendre, Losay, Touchot-Kone, Serraf, Belli and Piot297, Reference Losay, Touchot, Serraf, Litvinova, Lambert and Piot313, Reference Raisky, Bergoend, Agnoletti, Ou, Bonnet and Sidi383], and silent coronary obstructive lesions have a prevalence of 6–8% [Reference Legendre, Losay, Touchot-Kone, Serraf, Belli and Piot297, Reference Bonnet, Bonhoeffer, Piechaud, Aggoun, Sidi and Planche380]. Some patients are symptom-free, without diagnostic evidence of myocardial ischaemia, until they experience sudden cardiac events [Reference Angeli, Raisky, Bonnet, Sidi and Vouhé293, Reference Legendre, Losay, Touchot-Kone, Serraf, Belli and Piot297, Reference Vargo, Mavroudis, Stewart and Backer321, Reference Bonhoeffer, Bonnet, Piechaud, Stumper, Aggoun and Villain384, Reference Hutter, Bennink, Ay, Raes, Hitchcock and Meijboom385]. Coronary lesions were detected after a mean interval of 33 months. Because the lesions are usually progressive, coronary evaluation should be repeated regularly during late follow-up [Reference Angeli, Raisky, Bonnet, Sidi and Vouhé293].

Indications for selective coronary angiography or multislice CT angiography after an ASO include (i) the presence of electrocardiographic or echocardiographic signs suggestive of myocardial ischaemia at any time after the operation; (ii) the presence of unusual coronary patterns (e.g. coronary arteries coursing between the great arteries) or intraoperative difficulties in coronary transfer; and (iii) the use of a single-orifice technique for coronary reimplantation.

8.4.3 Coronary lesions: patient-related risk factors

  1. 1. Complex coronary anatomy. Early coronary lesions and coronary reoperation rates are more frequent in patients with complex or unusual coronary patterns [Reference Metton, Calvaruso, Gaudin, Mussa, Raisky and Bonnet142, Reference Rudra, Mavroudis, Backer, Kaushal, Russell and Stewart173, Reference Legendre, Losay, Touchot-Kone, Serraf, Belli and Piot297, Reference Vargo, Mavroudis, Stewart and Backer321, Reference Tamisier, Ouaknine, Pouard, Mauriat, Lefebvre and Sidi377, Reference Angeli, Formigari, Pace Napoleone, Oppido, Ragni and Picchio382]. Intramural origin or single-ostium looping was found to increase the risk for morbidity and mortality [Reference Pasquali, Hasselblad, Li, Kong and Sanders143]. Others did not find this correlation [Reference Thrupp, Gentles, Kerr and Finucane144, Reference Hutter, Bennink, Ay, Raes, Hitchcock and Meijboom385].

  2. 2. Bicuspid neoaortic valve. This condition might cause commissural malalignment and neoaortic root dilation; both conditions increase the risk for suboptimal coronary transfer [Reference Angeli, Gerelli, Beyler, Lamerain, Rochas and Bonnet386].

  3. 3. Relative position of the great arteries. Peculiar relationships between the reimplanted coronary arteries and the adjacent great arteries might lead to coronary compression [Reference Legendre, Losay, Touchot-Kone, Serraf, Belli and Piot297, Reference Bonnet, Bonhoeffer, Piechaud, Aggoun, Sidi and Planche380].

8.4.4 Coronary lesions: surgery-related risk factors

Technical details may impact on the need for reintervention after an ASO, and late coronary lesions may occur in all coronary patterns, even after the most straightforward initial operation [Reference Angeli, Raisky, Bonnet, Sidi and Vouhé293].

Mechanical reasons for coronary obstruction include proximal coronary artery compression, kinking and stretching. Various technical factors have been suspected: inadequate coronary transfer, type of coronary artery button technique, excessive use of fibrin glue and abnormal early fibrosis [Reference Rudra, Mavroudis, Backer, Kaushal, Russell and Stewart173, Reference Angeli, Raisky, Bonnet, Sidi and Vouhé293, Reference Legendre, Losay, Touchot-Kone, Serraf, Belli and Piot297, Reference Ou, Khraiche, Celermajer, Agnoletti, Le Quan Sang and Thalabard298, Reference Mavroudis, Stewart, Backer, Rudra, Vargo and Jacobs354].

8.4.5 Residual/recurrent coronary lesions: indications and management

Acute coronary insufficiency at the time of an ASO must be immediately addressed by revision of the anastomosis or by bypass grafting.

Reoperation in the event of late coronary insufficiency is indicated when myocardial ischaemia is demonstrated at myocardial imaging.

Different approaches have been used to manage post-ASO coronary lesions.

8.4.5.1 Surgical approaches

  1. 1. Coronary (ostial) patch angioplasty, using saphenous vein patch [Reference Angeli, Raisky, Bonnet, Sidi and Vouhé293, Reference Bonnet, Bonhoeffer, Sidi, Kachaner, Acar and Villain387, Reference Raanani, Kogan, Shapira, Sagie, Kornowsky and Vidne388], autologous or heterologous pericardial patch [Reference Vargo, Mavroudis, Stewart and Backer321, Reference Raisky, Bergoend, Agnoletti, Ou, Bonnet and Sidi383, Reference Bonnet, Bonhoeffer, Sidi, Kachaner, Acar and Villain387Reference Prifti, Bonacchi, Luisi and Vanini391] or the proximal segment of the right internal thoracic artery [Reference Jonsson, Jensen, Olsson, Holm and Liska392]. Adequate enlargement of a proximal coronary obstruction is achieved using a patch from the aorta, across the stenotic lesion, down to a normal coronary artery, restoring normal coronary perfusion [Reference Bonnet, Bonhoeffer, Sidi, Kachaner, Acar and Villain387, Reference Prêtre and Turina390, Reference Dion, Verhelst, Matta, Rousseau, Goenen and Chalant393]. Surgical arterioplasty might be contraindicated only when the left main coronary artery bifurcation is involved in the stenotic process [Reference Bergoend, Raisky, Degandt, Tamisier, Sidi and Vouhé389]. Coronary ostial patch angioplasty can be performed with low operative risk and high patency rate [Reference Bonhoeffer, Bonnet, Piechaud, Stumper, Aggoun and Villain384]. However, its long-term prognosis remains unknown.

  2. 2. Internal mammary artery grafting is technically feasible in most children, with satisfactory patency rates [Reference Rudra, Mavroudis, Backer, Kaushal, Russell and Stewart173, Reference Angeli, Raisky, Bonnet, Sidi and Vouhé293, Reference Mavroudis, Stewart, Backer, Rudra, Vargo and Jacobs354, Reference Bergoend, Raisky, Degandt, Tamisier, Sidi and Vouhé389, Reference Mavroudis, Backer, Duffy, Pahl and Wax394, Reference Mavroudis, Backer, Muster, Pahl, Sanders and Zales395]. Internal mammary grafting has been suggested only for more distal lesions, long and complete occlusions of the main stem or residual obstruction after primary surgical arterioplasty [Reference Bergoend, Raisky, Degandt, Tamisier, Sidi and Vouhé389]. However, blood flow through a mammary bypass may be inadequate, particularly when a large myocardial area must be revascularized [Reference Mavroudis, Backer, Duffy, Pahl and Wax394, Reference Mavroudis, Backer, Muster, Pahl, Sanders and Zales395].

8.4.5.2 Percutaneous transluminal coronary angioplasty in infants and young children

This procedure includes the possibility of inserting a coronary stent, as a first-time intervention to alleviate coronary stenosis, or after a previous coronary patch angioplasty operation [Reference Angeli, Formigari, Pace Napoleone, Oppido, Ragni and Picchio382, Reference Kampmann, Kuroczynski, Trubel, Knuf, Schneider and Heinemann396Reference El-Segaier, Lundin, Hochbergs, Jogi and Pesonen398]. In these rare patients a policy of routine angiographic evaluation of the coronary arteries within the first 2–3 years after repair is advisable [Reference Angeli, Formigari, Pace Napoleone, Oppido, Ragni and Picchio382].

ASO: arterial switch operation; CT: computed tomography; MRI: magnetic resonance imaging.

a Class of recommendation.

b Level of evidence.

c References.

Recommendations for reoperations for residual or recurrent coronary lesions

8.5 Other reoperations for late complications

8.5.1 Treatment of tracheobronchial compression

Tracheobronchial [Reference Robotin, Bruniaux, Serraf, Uva, Roussin and Lacour-Gayet402Reference Worsey, Pham, Newman, Park and del Nido404] and oesophageal [Reference McElhinney, Reddy, Reddy, Higgins and Hanley405] compression by vascular structures (aorta or pulmonary arteries) may develop after an ASO and require surgical relief.

The mechanism of ‘left bronchial compression’ relates to the posterior displacement of the ascending aorta behind the left pulmonary artery with either impingement of this vessel upon the left main bronchus or compression of the bronchus between the ascending and descending aorta (pincer effect) [Reference Worsey, Pham, Newman, Park and del Nido404, Reference Gandhi, Pigula and Siewers406]. Tracheography is a useful diagnostic method in cases with airway obstruction [Reference Toker, Tireli, Bostanci, Ozcan and Dayioglu403, Reference Corno, Giamberti, Giannico, Marino, Rossi and Marcelletti407]. Post-ASO left bronchial compression may be prevented using high transection on the great arteries and the Lecompte manoeuvre, leaving the anastomotic region above the left main bronchus level [Reference Toker, Tireli, Bostanci, Ozcan and Dayioglu403]. Compression of the left main bronchus by the posteriorly displaced aorta can be approached by an aortopexy procedure, through a left thoracotomy. In cases of persistent symptoms, tracheobronchomalacia must be ruled out [Reference Robotin, Bruniaux, Serraf, Uva, Roussin and Lacour-Gayet402].

8.5.2 Treatment of persistent pulmonary hypertension

Pulmonary vascular disease and pulmonary hypertension in TGA IVS are rare but severe [Reference Kirklin, Blackstone, Tchervenkov and Castaneda138, Reference Haas, Wottke, Poppert and Meisner316, Reference Daebritz, Nollert, Sachweh, Engelhardt, von Bernuth and Messmer351, Reference Kumar, Taylor, Sandor and Patterson408] conditions that can occur even in patients undergoing an ASO before the age of 1 month [Reference Losay, Touchot, Serraf, Litvinova, Lambert and Piot313]. Medical therapy should first be provided [Reference Damen and Hitchcock409], but for the most resilient cases, surgical management may be attempted, using either blade atrial septostomy [Reference Kerstein, Levy, Hsu, Hordof, Gersony and Barst410] or a Potts anastomosis [Reference Angeli, Raisky, Bonnet, Sidi and Vouhé293, Reference Blanc, Vouhé and Bonnet411]. The goal of the latter procedure is to decrease right ventricular afterload, which improves right ventricular function and potentially prevents syncope and sudden death [Reference Blanc, Vouhé and Bonnet411].

8.5.3 Treatment of transposition of the great arteries with aortopulmonary collaterals

Aortopulmonary collaterals have long been known to coexist with TGA [Reference Aziz, Paul and Rowe412]. They present angiographically as enlarged bronchial arteries [Reference Wernovsky, Bridges, Mandell, Castaneda and Perry413]. Significant collateral flow may first become manifest during surgical correction, when significant left atrial or pulmonary artery return is noticed during CPB; intraoperative pulmonary haemorrhage has also been reported [Reference Golej, Trittenwein, Marx and Schlemmer414]. Coil embolization can be carried out either before [Reference Jowett, Derrick, Tsang and Marek415] or after [Reference Wernovsky, Bridges, Mandell, Castaneda and Perry413Reference Irving and Chaudhari416] an ASO, with complete occlusion of the vessels and no significant complications [Reference Wernovsky, Bridges, Mandell, Castaneda and Perry413, Reference Golej, Trittenwein, Marx and Schlemmer414, Reference Irving and Chaudhari416, Reference Santoro, Carrozza, Russo and Calabro417].

8.5.4 Treatment of residual mitral regurgitation

Mitral valve reoperations are rarely necessary for ischaemic mitral regurgitation or residual mitral valve malformation (mitral cleft) [Reference Raju, Burkhart, Durham, Eidem, Phillips and Li169, Reference Angeli, Raisky, Bonnet, Sidi and Vouhé293, Reference Losay, Touchot, Serraf, Litvinova, Lambert and Piot313].

Conflict of interest: none declared.

Footnotes

Disclosure: The members of the Task Force have provided disclosure statements of all relationships that might be perceived as real or potential sources of conflicts of interest. These disclosure forms are kept on file at the headquarters of the EACTS/AEPC. The Committee report received its entire financial support from the EACTS and AEPC, without any involvement of the pharmaceutical, device or surgical industries

References

1. Sousa-Uva, M, Head, SJ, Thielmann, M, Cardillo, G, Benedetto, U, Czerny, M, et al. Methodology manual for European Association for Cardio-Thoracic Surgery (EACTS) clinical guidelines. Eur J Cardiothorac Surg 2015; 48: 809816.Google ScholarPubMed
2. Marek, J, Tomek, V, Skovranek, J, Povysilova, V, Samanek, M. Prenatal ultrasound screening of congenital heart disease in an unselected national population: a 21-year experience. Heart 2011; 97: 124130.CrossRefGoogle Scholar
3. Fricke, TA, D’Udekem, Y, Richardson, M, Thuys, C, Dronavalli, M, Ramsay, JM, et al. Outcomes of the arterial switch operation for transposition of the great arteries: 25 years of experience. Ann Thorac Surg 2012; 94: 139145.CrossRefGoogle ScholarPubMed
4. Kempny, A, Wustmann, K, Borgia, F, Dimopoulos, K, Uebing, A, Li, W, et al. Outcome in adult patients after arterial switch operation for transposition of the great arteries. Int J Cardiol 2013; 167: 25882593.CrossRefGoogle ScholarPubMed
5. Lim, HG, Kim, WH, Lee, JR, Kim, YJ. Long-term results of the arterial switch operation for ventriculo-arterial discordance. Eur J Cardiothorac Surg 2013; 43: 325334.Google Scholar
6. Oda, S, Nakano, T, Sugiura, J, Fusazaki, N, Ishikawa, S, Kado, H. Twenty-eight years’ experience of arterial switch operation for transposition of the great arteries in a single institution. Eur J Cardiothorac Surg 2012; 42: 674679.Google Scholar
7. Stoica, S, Carpenter, E, Campbell, D, Mitchell, M, da Cruz, E, Ivy, D, et al. Morbidity of the arterial switch operation. Ann Thorac Surg 2012; 93: 19771983.CrossRefGoogle ScholarPubMed
8. Jatene, AD, Fontes, VF, Paulista, PP, Souza, LC, Neger, F, Galantier, M, et al. Anatomic correction of transposition of the great vessels. J Thorac Cardiovasc Surg 1976; 72: 364370.CrossRefGoogle ScholarPubMed
9. Bonnet, D, Coltri, A, Butera, G, Fermont, L, Le Bidois, J, Kachaner, J, et al. Detection of transposition of the great arteries in fetuses reduces neonatal morbidity and mortality. Circulation 1999; 99: 916918.Google Scholar
10. Pascal, CJ, Huggon, I, Sharland, GK, Simpson, JM. An echocardiographic study of diagnostic accuracy, prediction of surgical approach, and outcome for fetuses diagnosed with discordant ventriculo-arterial connections. Cardiol Young 2007; 17: 528534.Google Scholar
11. Perolo, A, Prandstraller, D, Ghi, T, Gargiulo, G, Leone, O, Bovicelli, L, et al. Diagnosis and management of fetal cardiac anomalies: 10 years of experience at a single institution. Ultrasound Obstet Gynecol 2001; 18: 615618.Google Scholar
12. Sivanandam, S, Glickstein, JS, Printz, BF, Allan, LD, Altmann, K, Solowiejczyk, DE, et al. Prenatal diagnosis of conotruncal malformations: diagnostic accuracy, outcome, chromosomal abnormalities, and extracardiac anomalies. Am J Perinatol 2006; 23: 241245.Google Scholar
13. Allan, LD, Sharland, GK, Milburn, A, Lockhart, SM, Groves, AM, Anderson, RH, et al. Prospective diagnosis of 1006 consecutive cases of congenital heart disease in the fetus. J Am Coll Cardiol 1994; 23: 14521458.CrossRefGoogle Scholar
14. Bull, C. Current and potential impact of fetal diagnosis on prevalence and spectrum of serious congenital heart disease at term in the UK. British Paediatric Cardiac Association. Lancet 1999; 354: 12421247.CrossRefGoogle ScholarPubMed
15. Jaeggi, ET, Sholler, GF, Jones, OD, Cooper, SG. Comparative analysis of pattern, management and outcome of pre- versus postnatally diagnosed major congenital heart disease: a population-based study. Ultrasound Obstet Gynecol 2001; 17: 380385.CrossRefGoogle ScholarPubMed
16. Sharland, G. Routine fetal cardiac screening: what are we doing and what should we do? Prenat Diagn 2004; 24: 11231129.Google Scholar
17. Wigton, TR, Sabbagha, RE, Tamura, RK, Cohen, L, Minogue, JP, Strasburger, JF. Sonographic diagnosis of congenital heart disease: comparison between the four-chamber view and multiple cardiac views. Obstet Gynecol 1993; 82: 219224.Google Scholar
18. Khoshnood, B, Lelong, N, Houyel, L, Thieulin, AC, Jouannic, JM, Magnier, S, et al. Prevalence, timing of diagnosis and mortality of newborns with congenital heart defects: a population-based study. Heart 2012; 98: 16671673.Google Scholar
19. Escobar-Diaz, MC, Freud, LR, Bueno, A, Brown, DW, Friedman, KG, Schidlow, D, et al. Prenatal diagnosis of transposition of the great arteries over a 20-year period: improved but imperfect. Ultrasound Obstet Gynecol 2015; 45: 678682.Google Scholar
20. van Velzen, CL, Haak, MC, Reijnders, G, Rijlaarsdam, ME, Bax, CJ, Pajkrt, E, et al. Prenatal detection of transposition of the great arteries reduces mortality and morbidity. Ultrasound Obstet Gynecol 2015; 45: 320325.Google Scholar
21. International Society of Ultrasound in Obstet Gynecol Education Committee. Cardiac screening examination of the fetus: guidelines for performing the ‘basic’ and ‘extended basic’ cardiac scan. Ultrasound Obstet Gynecol 2006; 27: 107113.Google Scholar
22. National Institute for Health and Clinical Excellence. Antenatal Care – Routine Care for the Healthy Pregnant Woman. Clinical guideline. NICE, London: RCOG Press; 2008.Google Scholar
23. NHS. Fetal Anomaly Screening Programme 18 + 0 to 20 + 6 Weeks Fetal Anomaly Scan. National Standards and Guidance for England. London: NHS Screening Programs; 2015.Google Scholar
24. Hunter, S, Heads, A, Wyllie, J, Robson, S. Prenatal diagnosis of congenital heart disease in the northern region of England: benefits of a training programme for obstetric ultrasonographers. Heart 2000; 84: 294298.Google Scholar
25. McBrien, A, Sands, A, Craig, B, Dornan, J, Casey, F. Impact of a regional training program in fetal echocardiography for sonographers on the antenatal detection of major congenital heart disease. Ultrasound Obstet Gynecol 2010; 36: 279284.Google Scholar
26. Pezard, P, Bonnemains, L, Boussion, F, Sentilhes, L, Allory, P, Lepinard, C, et al. Influence of ultrasonographers training on prenatal diagnosis of congenital heart diseases: a 12-year population-based study. Prenat Diagn 2008; 28: 10161022.Google Scholar
27. Sharland, GK, Allan, LD. Screening for congenital heart disease prenatally. Results of a 2 1/2-year study in the South East Thames region. Br J Obstet Gynaecol 1992; 99: 220225.CrossRefGoogle ScholarPubMed
28. Tegnander, E, Eik-Nes, SH. The examiner’s ultrasound experience has a significant impact on the detection rate of congenital heart defects at the second-trimester fetal examination. Ultrasound Obstet Gynecol 2006; 28: 814.CrossRefGoogle Scholar
29. British Congenital Cardiac Association. Fetal Cardiology Standards. London: British Congenital Cardiac Association; 2012.Google Scholar
30. Allan, L, Dangel, J, Fesslova, V, Marek, J, Mellander, M, Oberhansli, I, et al. Recommendations for the practice of fetal cardiology in Europe. Cardiol Young 2004; 14: 109114.Google Scholar
31. Copel, JA, Pilu, G, Kleinman, CS. Extracardiac anomalies and congenital heart disease. Semin Perinatol 1993; 17: 89105.Google Scholar
32. Paladini, D, Calabro, R, Palmieri, S, D’andrea, T. Prenatal diagnosis of congenital heart disease and fetal karyotyping. Obstet Gynecol 1993; 81: 679682.Google Scholar
33. Raymond, FL, Simpson, JM, Sharland, GK, Ogilvie Mackie, CM. Fetal echocardiography as a predictor of chromosomal abnormality. Lancet 1997; 350: 930.CrossRefGoogle ScholarPubMed
34. Tennstedt, C, Chaoui, R, Korner, H, Dietel, M. Spectrum of congenital heart defects and extracardiac malformations associated with chromosomal abnormalities: results of a seven year necropsy study. Heart 1999; 82: 3439.Google Scholar
35. Jouannic, JM, Gavard, L, Fermont, L, Le Bidois, J, Parat, S, Vouhé, PR, et al. Sensitivity and specificity of prenatal features of physiological shunts to predict neonatal clinical status in transposition of the great arteries. Circulation 2004; 110: 17431746.CrossRefGoogle ScholarPubMed
36. Maeno, YV, Kamenir, SA, Sinclair, B, van der Velde, ME, Smallhorn, JF, Hornberger, LK. Prenatal features of ductus arteriosus constriction and restrictive foramen ovale in d-transposition of the great arteries. Circulation 1999; 99: 12091214.Google Scholar
37. Punn, R, Silverman, NH. Fetal predictors of urgent balloon atrial septostomy in neonates with complete transposition. J Am Soc Echocardiogr 2011; 24: 425430.CrossRefGoogle ScholarPubMed
38. Fuchs, IB, Muller, H, Abdul-Khaliq, H, Harder, T, Dudenhausen, JW, Henrich, W. Immediate and long-term outcomes in children with prenatal diagnosis of selected isolated congenital heart defects. Ultrasound Obstet Gynecol 2007; 29: 3843.Google Scholar
39. Khoshnood, B, De Vigan, C, Vodovar, V, Goujard, J, Lhomme, A, Bonnet, D, et al. Trends in prenatal diagnosis, pregnancy termination, and perinatal mortality of newborns with congenital heart disease in France, 1983-2000: a population-based evaluation. Pediatrics 2005; 115: 95101.Google Scholar
40. Bartlett, JM, Wypij, D, Bellinger, DC, Rappaport, LA, Heffner, LJ, Jonas, RA, et al. Effect of prenatal diagnosis on outcomes in D-transposition of the great arteries. Pediatrics 2004; 113: E335E340.Google Scholar
41. Kumar, RK, Newburger, JW, Gauvreau, K, Kamenir, SA, Hornberger, LK. Comparison of outcome when hypoplastic left heart syndrome and transposition of the great arteries are diagnosed prenatally versus when diagnosis of these two conditions is made only postnatally. Am J Cardiol 1999; 83: 16491653.Google Scholar
42. Levey, A, Glickstein, JS, Kleinman, CS, Levasseur, SM, Chen, J, Gersony, WM, et al. The impact of prenatal diagnosis of complex congenital heart disease on neonatal outcomes. Pediatr Cardiol 2010; 31: 587597.Google Scholar
43. Verheijen, PM, Lisowski, LA, Stoutenbeek, P, Hitchcock, JF, Bennink, GB, Meijboom, EJ. Lactacidosis in the neonate is minimized by prenatal detection of congenital heart disease. Ultrasound Obstet Gynecol 2002; 19: 552555.Google Scholar
44. Hellstrom-Westas, L, Hanseus, K, Jogi, P, Lundstrom, NR, Svenningsen, N. Long-distance transports of newborn infants with congenital heart disease. Pediatr Cardiol 2001; 22: 380384.CrossRefGoogle ScholarPubMed
45. Mirlesse, V, Cruz, A, Le Bidois, J, Diallo, P, Fermont, L, Kieffer, F, et al. Perinatal management of fetal cardiac anomalies in a specialized obstetric-pediatrics center. Am J Perinatol 2001; 18: 363371.Google Scholar
46. Jegatheeswaran, A, Oliveira, C, Batsos, C, Moon-Grady, AJ, Silverman, NH, Hornberger, LK, et al. Costs of prenatal detection of congenital heart disease. Am J Cardiol 2011; 108: 18081814.Google Scholar
47. Landis, BJ, Levey, A, Levasseur, SM, Glickstein, JS, Kleinman, CS, Simpson, LL, et al. Prenatal diagnosis of congenital heart disease and birth outcomes. Pediatr Cardiol 2013; 34: 597605.Google Scholar
48. Eckersley, L, Sadler, L, Parry, E, Finucane, K, Gentles, TL. Timing of diagnosis affects mortality in critical congenital heart disease. Arch Dis Child 2016; 101: 516520.CrossRefGoogle ScholarPubMed
49. McMahon, CJ, el Said, HG, Feltes, TF, Watrin, CH, Hess, BA, Fraser, CD Jr. Preoperative identification of coronary arterial anatomy in complete transposition, and outcome after the arterial switch operation. Cardiol Young 2002; 12: 240247.Google Scholar
50. Pasquini, L, Sanders, SP, Parness, IA, Wernovsky, G, Mayer, JE Jr, Van der Velde, ME, et al. Coronary echocardiography in 406 patients with d-loop transposition of the great arteries. J Am Coll Cardiol 1994; 24: 763768.Google Scholar
51. Studer, MA, Smith, AE, Lustik, MB, Carr, MR. Newborn pulse oximetry screening to detect critical congenital heart disease. J Pediatr 2014; 164: 5059.e1-2.Google Scholar
52. Lopes, LM, Kawano, C, Cristovao, SA, Nagamatsu, CT, Fonseca, L, Furlanetto, BH, et al. Balloon atrial septostomy guided by echocardiography in a neonatal intensive care unit. Arq Bras Cardiol 2010; 95: 153158.Google Scholar
53. Martin, AC, Rigby, ML, Penny, DJ, Redington, AN. Bedside balloon atrial septostomy on neonatal units. Arch Dis Child Fetal Neonatal Ed 2003; 88: F339F340.CrossRefGoogle ScholarPubMed
54. Zellers, TM, Dixon, K, Moake, L, Wright, J, Ramaciotti, C. Bedside balloon atrial septostomy is safe, efficacious, and cost-effective compared with septostomy performed in the cardiac catheterization laboratory. Am J Cardiol 2002; 89: 613615.CrossRefGoogle ScholarPubMed
55. Browning Carmo, KA, Barr, P, West, M, Hopper, NW, White, JP, Badawi, N. Transporting newborn infants with suspected duct dependent congenital heart disease on low-dose prostaglandin E1 without routine mechanical ventilation. Arch Dis Child Fetal Neonatal Ed 2007; 92: F117F119.CrossRefGoogle ScholarPubMed
56. Meckler, GD, Lowe, C. To intubate or not to intubate? Transporting infants on prostaglandin E1. Pediatrics 2009; 123: E25E30.Google Scholar
57. Barton, KP, Munoz, R, Morell, VO, Chrysostomou, C. Dexmedetomidine as the primary sedative during invasive procedures in infants and toddlers with congenital heart disease. Pediatr Crit Care Med 2008; 9: 612615.Google Scholar
58. Finan, E, Mak, W, Bismilla, Z, McNamara, PJ. Early discontinuation of intravenous prostaglandin E1 after balloon atrial septostomy is associated with an increased risk of rebound hypoxemia. J Perinatol 2008; 28: 341346.Google Scholar
59. Oxenius, A, Hug, MI, Dodge-Khatami, A, Cavigelli-Brunner, A, Bauersfeld, U, Balmer, C. Do predictors exist for a successful withdrawal of preoperative prostaglandin E(1) from neonates with d-transposition of the great arteries and intact ventricular septum? Pediatr Cardiol 2010; 31: 11981202.Google Scholar
60. Lim, DS, Kulik, TJ, Kim, DW, Charpie, JR, Crowley, DC, Maher, KO. Aminophylline for the prevention of apnea during prostaglandin E1 infusion. Pediatrics 2003; 112: E27E29.Google Scholar
61. Roofthooft, MT, Bergman, KA, Waterbolk, TW, Ebels, T, Bartelds, B, Berger, RM. Persistent pulmonary hypertension of the newborn with transposition of the great arteries. Ann Thorac Surg 2007; 83: 14461450.Google Scholar
62. Newfeld, EA, Paul, MM, Muster, AJ, Idriss, FS. Pulmonary vascular disease in complete transposition of the great arteries: a study of 200 patients. Am J Cardiol 1974; 34: 7582.Google Scholar
63. Fan, H, Hu, S, Zheng, Z, Li, S, Zhang, Y, Pan, X, et al. Do patients with complete transposition of the great arteries and severe pulmonary hypertension benefit from an arterial switch operation? Ann Thorac Surg 2011; 91: 181186.Google Scholar
64. Chang, AC, Wernovsky, G, Kulik, TJ, Jonas, RA, Wessel, DL. Management of the neonate with transposition of the great arteries and persistent pulmonary hypertension. Am J Cardiol 1991; 68: 12531255.CrossRefGoogle ScholarPubMed
65. El-Segaier, M, Hellstrom-Westas, L, Wettrell, G. Nitric oxide in neonatal transposition of the great arteries. Acta Paediatr 2005; 94: 912916.CrossRefGoogle ScholarPubMed
66. Goissen, C, Ghyselen, L, Tourneux, P, Krim, G, Storme, L, Bou, P, et al. Persistent pulmonary hypertension of the newborn with transposition of the great arteries: successful treatment with bosentan. Eur J Pediatr 2008; 167: 437440.CrossRefGoogle ScholarPubMed
67. Jaillard, S, Belli, E, Rakza, T, Larrue, B, Magnenant, E, Rey, C, et al. Preoperative ECMO in transposition of the great arteries with persistent pulmonary hypertension. Ann Thorac Surg 2005; 79: 21552158.CrossRefGoogle ScholarPubMed
68. Luciani, GB, Chang, AC, Starnes, VA. Surgical repair of transposition of the great arteries in neonates with persistent pulmonary hypertension. Ann Thorac Surg 1996; 61: 800805.Google Scholar
69. Mair, DD, Ritter, DG. Factors influencing intercirculatory mixing in patients with complete transposition of the great arteries. Am J Cardiol 1972; 30: 653658.Google Scholar
70. Shaher, RM. The haemodynamics of complete transposition of the great vessels. Br Heart J 1964; 26: 343353.Google Scholar
71. Shiao, SY, Ou, CN. Validation of oxygen saturation monitoring in neonates. Am J Crit Care 2007; 16: 168178.Google Scholar
72. Marino, BS, Bird, GL, Wernovsky, G. Diagnosis and management of the newborn with suspected congenital heart disease. Clin Perinatol 2001; 28: 91136.Google Scholar
73. Howley, LW, Kaufman, J, Wymore, E, Thureen, P, Magouirk, JK, McNair, B, et al. Enteral feeding in neonates with prostaglandin-dependent congenital cardiac disease: international survey on current trends and variations in practice. Cardiol Young 2012; 22: 121127.Google Scholar
74. Willis, L, Thureen, P, Kaufman, J, Wymore, E, Skillman, H, da Cruz, E. Enteral feeding in prostaglandin-dependent neonates: is it a safe practice? J Pediatr 2008; 153: 867869.Google Scholar
75. Sables-Baus, S, Kaufman, J, Cook, P, da Cruz, EM. Oral feeding outcomes in neonates with congenital cardiac disease undergoing cardiac surgery. Cardiol Young 2012; 22: 4248.Google Scholar
76. Davis, D, Davis, S, Cotman, K, Worley, S, Londrico, D, Kenny, D, et al. Feeding difficulties and growth delay in children with hypoplastic left heart syndrome versus d-transposition of the great arteries. Pediatr Cardiol 2008; 29: 328333.Google Scholar
77. Natarajan, G, Reddy Anne, S, Aggarwal, S. Enteral feeding of neonates with congenital heart disease. Neonatology 2010; 98: 330336.Google Scholar
78. Soongswang, J, Adatia, I, Newman, C, Smallhorn, JF, Williams, WG, Freedom, RM. Mortality in potential arterial switch candidates with transposition of the great arteries. J Am Coll Cardiol 1998; 32: 753757.Google Scholar
79. Ades, A, Johnson, BA, Berger, S. Management of low birth weight infants with congenital heart disease. Clin Perinatol 2005; 32: 9991015; x-xi. .Google Scholar
80. Hickey, EJ, Nosikova, Y, Zhang, H, Caldarone, CA, Benson, L, Redington, A, et al. Very low-birth-weight infants with congenital cardiac lesions: is there merit in delaying intervention to permit growth and maturation? J Thorac Cardiovasc Surg 2012; 143: 126136.Google Scholar
81. Reddy, VM. Low birth weight and very low birth weight neonates with congenital heart disease: timing of surgery, reasons for delaying or not delaying surgery. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2013; 16: 1320.Google Scholar
82. Curzon, CL, Milford-Beland, S, Li, JS, O’brien, SM, Jacobs, JP, Jacobs, ML, et al. Cardiac surgery in infants with low birth weight is associated with increased mortality: analysis of the Society of Thoracic Surgeons Congenital Heart Database. J Thorac Cardiovasc Surg 2008; 135: 546551.Google Scholar
83. Kansy, A, Tobota, Z, Maruszewski, P, Maruszewski, B. Analysis of 14,843 neonatal congenital heart surgical procedures in the European Association for Cardiothoracic Surgery Congenital Database. Ann Thorac Surg 2010; 89: 12551259.Google Scholar
84. Reddy, VM, McElhinney, DB, Sagrado, T, Parry, AJ, Teitel, DF, Hanley, FL. Results of 102 cases of complete repair of congenital heart defects in patients weighing 700 to 2500 grams. J Thorac Cardiovasc Surg 1999; 117: 324331.Google Scholar
85. Roussin, R, Belli, E, Bruniaux, J, Demontoux, S, Touchot, A, Planche, C, et al. Surgery for transposition of the great arteries in neonates weighing less than 2000 grams: a consecutive series of 25 patients. Ann Thorac Surg 2007; 83: 173177.Google Scholar
86. Azakie, A, Johnson, NC, Anagnostopoulos, PV, Egrie, GD, Lavrsen, MJ, Sapru, A. Cardiac surgery in low birth weight infants: current outcomes. Interact CardioVasc Thorac Surg 2011; 12: 409413.Google Scholar
87. Rios, R, Dummer, KB, Overman, DM. Successful staged surgical repair using rapid pulmonary artery banding in a very-low-birth-weight premature infant who had d-transposition of the great arteries with an intact ventricular septum. Pediatr Cardiol 2013; 34: 19351937.Google Scholar
88. Castaneda, AR, Norwood, WI, Jonas, RA, Colon, SD, Sanders, SP, Lang, P. Transposition of the great arteries and intact ventricular septum: anatomical repair in the neonate. Ann Thorac Surg 1984; 38: 438443.Google Scholar
89. Quaegebeur, JM, Rohmer, J, Ottenkamp, J, Buis, T, Kirklin, JW, Blackstone, EH, et al. The arterial switch operation. An eight-year experience. J Thorac Cardiovasc Surg 1986; 92: 361384.Google Scholar
90. Sarris, GE, Chatzis, AC, Giannopoulos, NM, Kirvassilis, G, Berggren, H, Hazekamp, M, et al. The arterial switch operation in Europe for transposition of the great arteries: a multi-institutional study from the European Congenital Heart Surgeons Association. J Thorac Cardiovasc Surg 2006; 132: 633639.Google Scholar
91. Danford, DA, Huhta, JC, Gutgesell, HP. Left ventricular wall stress and thickness in complete transposition of the great arteries. Implications for surgical intervention. J Thorac Cardiovasc Surg 1985; 89: 610615.Google Scholar
92. Rudolph, AM. Myocardial growth before and after birth: clinical implications. Acta Paediatr 2000; 89: 129133.Google Scholar
93. Duncan, BW, Poirier, NC, Mee, RB, Drummond-Webb, JJ, Qureshi, A, Mesia, CI, et al. Selective timing for the arterial switch operation. Ann Thorac Surg 2004; 77: 16911696.Google Scholar
94. Chasovskyi, K, Fedevych, O, Vorobiova, G, Zhovnir, V, Maksimenko, A, Boychenko, O, et al. Arterial switch operation in the first hours of life using autologous umbilical cord blood. Ann Thorac Surg 2012; 93: 15711576.Google Scholar
95. Nevvazhay, T, Chernogrivov, A, Biryukov, E, Biktasheva, L, Karchevskaya, K, Sulejmanov, S, et al. Arterial switch in the first hours of life: no need for Rashkind septostomy? Eur J Cardiothorac Surg 2012; 42: 520523.Google Scholar
96. Bisoi, AK, Sharma, P, Chauhan, S, Reddy, SM, Das, S, Saxena, A, et al. Primary arterial switch operation in children presenting late with d-transposition of great arteries and intact ventricular septum. When is it too late for a primary arterial switch operation? Eur J Cardiothorac Surg 2010; 38: 707713.CrossRefGoogle ScholarPubMed
97. Davis, AM, Wilkinson, JL, Karl, TR, Mee, RB. Transposition of the great arteries with intact ventricular septum. Arterial switch repair in patients 21 days of age or older. J Thorac Cardiovasc Surg 1993; 106: 111115.Google Scholar
98. Edwin, F, Mamorare, H, Brink, J, Kinsley, R. Primary arterial switch operation for transposition of the great arteries with intact ventricular septum--is it safe after three weeks of age? Interact CardioVasc Thorac Surg 2010; 11: 641644.Google Scholar
99. Foran, JP, Sullivan, ID, Elliott, MJ, de Leval, MR. Primary arterial switch operation for transposition of the great arteries with intact ventricular septum in infants older than 21 days. J Am Coll Cardiol 1998; 31: 883889.CrossRefGoogle Scholar
100. Kang, N, de Leval, MR, Elliott, M, Tsang, V, Kocyildirim, E, Sehic, I, et al. Extending the boundaries of the primary arterial switch operation in patients with transposition of the great arteries and intact ventricular septum. Circulation 2004; 110: 123127.Google Scholar
101. D’Udekem, Y, Cheung, M, Butt, W, Shann, F, Brizard, CP. Transposition with intact septum diagnosed at nine months: arterial switch? Asian Cardiovasc Thorac Ann 2012; 20: 333334.Google Scholar
102. Di Donato, RM, Fujii, AM, Jonas, RA, Castaneda, AR. Age-dependent ventricular response to pressure overload. Considerations for the arterial switch operation. J Thorac Cardiovasc Surg 1992; 104: 713722.Google Scholar
103. Bisoi, AK, Malankar, D, Chauhan, S, Das, S, Ray, R, Das, P. An electron microscopic study of left ventricular regression in children with transposition of great arteries. Interact CardioVasc Thorac Surg 2010; 11: 768772.Google Scholar
104. Rudolph, AM. The fetal circulation and its adjustments after birth in congenital heart disease. UCLA Forum Med Sci 1970; 10: 105118.Google Scholar
105. Lacour-Gayet, F, Piot, D, Zoghbi, J, Serraf, A, Gruber, P, Mace, L, et al. Surgical management and indication of left ventricular retraining in arterial switch for transposition of the great arteries with intact ventricular septum. Eur J Cardiothorac Surg 2001; 20: 824829.Google Scholar
106. Robinson, PJ, Wyse, RK, Macartney, FJ. Left ventricular outflow tract obstruction in complete transposition of the great arteries with intact ventricular septum. A cross sectional echocardiography study. Br Heart J 1985; 54: 201208.Google Scholar
107. Bano-Rodrigo, A, Quero-Jimenez, M, Moreno-Granado, F, Gamallo-Amat, C. Wall thickness of ventricular chambers in transposition of the great arteries: surgical implications. J Thorac Cardiovasc Surg 1980; 79: 592597.Google Scholar
108. Huhta, JC, Edwards, WD, Feldt, RH, Puga, FJ. Left ventricular wall thickness in complete transposition of the great arteries. J Thorac Cardiovasc Surg 1982; 84: 97101.CrossRefGoogle ScholarPubMed
109. Maroto, E, Fouron, JC, Douste-Blazy, MY, Carceller, AM, van Doesburg, N, Kratz, C, et al. Influence of age on wall thickness, cavity dimensions and myocardial contractility of the left ventricle in simple transposition of the great arteries. Circulation 1983; 67: 13111317.Google Scholar
110. Smith, A, Wilkinson, JL, Arnold, R, Dickinson, DF, Anderson, RH. Growth and development of ventricular walls in complete transposition of the great arteries with intact septum (simple transposition). Am J Cardiol 1982; 49: 362368.CrossRefGoogle ScholarPubMed
111. Iyer, KS, Sharma, R, Kumar, K, Bhan, A, Kothari, SS, Saxena, A, et al. Serial echocardiography for decision making in rapid two-stage arterial switch operation. Ann Thorac Surg 1995; 60: 658664.Google Scholar
112. Sidi, D, Planche, C, Kachaner, J, Bruniaux, J, Villain, E, Le Bidois, J, et al. Anatomic correction of simple transposition of the great arteries in 50 neonates. Circulation 1987; 75: 429435.Google Scholar
113. Dabritz, S, Engelhardt, W, von Bernuth, G, Messmer, BJ. Trial of pulmonary artery banding: a diagnostic criterion for ‘one-stage’ arterial switch in simple transposition of the great arteries beyond the neonatal period. Eur J Cardiothorac Surg 1997; 11: 112116.CrossRefGoogle ScholarPubMed
114. Yacoub, MH, Radley-Smith, R, Maclaurin, R. Two-stage operation for anatomical correction of transposition of the great arteries with intact interventricular septum. Lancet 1977; 1: 12751278.Google Scholar
115. Borow, KM, Arensman, FW, Webb, C, Radley-Smith, R, Yacoub, MH. Assessment of left ventricular contractile state after anatomic correction of transposition of the great arteries. Circulation 1984; 69: 106112.Google Scholar
116. Sievers, HH, Lange, PE, Onnasch, DG, Radley-Smith, R, Yacoub, MH, Heintzen, PH, et al. Influence of the two-stage anatomic correction of simple transposition of the great arteries on left ventricular function. Am J Cardiol 1985; 56: 514519.Google Scholar
117. Jonas, RA, Giglia, TM, Sanders, SP, Wernovsky, G, Nadal-Ginard, B, Mayer, JE Jr, et al. Rapid, two-stage arterial switch for transposition of the great arteries and intact ventricular septum beyond the neonatal period. Circulation 1989; 80: I203I208.Google Scholar
118. Boutin, C, Jonas, RA, Sanders, SP, Wernovsky, G, Mone, SM, Colan, SD. Rapid two-stage arterial switch operation. Acquisition of left ventricular mass after pulmonary artery banding in infants with transposition of the great arteries. Circulation 1994; 90: 13041309.Google Scholar
119. Wernovsky, G, Giglia, TM, Jonas, RA, Mone, SM, Colan, SD, Wessel, DL. Course in the intensive care unit after ‘preparatory’ pulmonary artery banding and aortopulmonary shunt placement for transposition of the great arteries with low left ventricular pressure. Circulation 1992; 86: 133139.Google Scholar
120. Parker, NM, Zuhdi, M, Kouatli, A, Baslaim, G. Late presenters with dextro-transposition of great arteries and intact ventricular septum: to train or not to train the left ventricle for arterial switch operation? Congenit Heart Dis 2009; 4: 424432.Google Scholar
121. Al Qethamy, HO, Aizaz, K, Aboelnazar, SA, Hijab, S, Al Faraidi, Y. Two-stage arterial switch operation: is late ever too late? Asian Cardiovasc Thorac Ann 2002; 10: 235239.Google Scholar
122. Helvind, MH, McCarthy, JF, Imamura, M, Prieto, L, Sarris, GE, Drummond-Webb, JJ, et al. Ventriculo-arterial discordance: switching the morphologically left ventricle into the systemic circulation after 3 months of age. Eur J Cardiothorac Surg 1998; 14: 173178.CrossRefGoogle ScholarPubMed
123. Ilbawi, MN, Idriss, FS, DeLeon, SY, Muster, AJ, Gidding, SS, Duffy, CE, et al. Preparation of the left ventricle for anatomical correction in patients with simple transposition of the great arteries. Surgical guidelines. J Thorac Cardiovasc Surg 1987; 94: 8794.Google Scholar
124. Bernhard, A, Yacoub, M, Regensburger, D, Sievers, HH, Smith, RR, Stephan, E, et al. Further experience with the two-stage anatomic correction of simple transposition of the great arteries. Thorac Cardiovasc Surg 1981; 29: 138142.Google Scholar
125. Sivakumar, K, Francis, E, Krishnan, P, Shahani, J. Ductal stenting retrains the left ventricle in transposition of great arteries with intact ventricular septum. J Thorac Cardiovasc Surg 2006; 132: 10811086.Google Scholar
126. Harinck, E, Van Mill, GJ, Ross, D, Brom, AG. Anatomical correction of transposition of great arteries with persistent ductus arteriosus. One year after operation. Br Heart J 1980; 43: 9598.Google Scholar
127. Boutin, C, Wernovsky, G, Sanders, SP, Jonas, RA, Castaneda, AR, Colan, SD. Rapid two-stage arterial switch operation. Evaluation of left ventricular systolic mechanics late after an acute pressure overload stimulus in infancy. Circulation 1994; 90: 12941303.Google Scholar
128. Colan, SD, Boutin, C, Castaneda, AR, Wernovsky, G. Status of the left ventricle after arterial switch operation for transposition of the great arteries. Hemodynamic and echocardiographic evaluation. J Thorac Cardiovasc Surg 1995; 109: 311321.Google Scholar
129. Wernovsky, G, Wypij, D, Jonas, RA, Mayer, JE Jr, Hanley, FL, Hickey, PR, et al. Postoperative course and hemodynamic profile after the arterial switch operation in neonates and infants. A comparison of low-flow cardiopulmonary bypass and circulatory arrest. Circulation 1995; 92: 22262235.Google Scholar
130. Nakazawa, M, Oyama, K, Imai, Y, Nojima, K, Aotsuka, H, Satomi, G, et al. Criteria for two-staged arterial switch operation for simple transposition of great arteries. Circulation 1988; 78: 124131.CrossRefGoogle ScholarPubMed
131. Bellinger, DC, Wypij, D, duPlessis, AJ, Rappaport, LA, Jonas, RA, Wernovsky, G, et al. Neurodevelopmental status at eight years in children with dextro-transposition of the great arteries: the Boston Circulatory Arrest Trial. J Thorac Cardiovasc Surg 2003; 126: 13851396.Google Scholar
132. Karl, TR, Hall, S, Ford, G, Kelly, EA, Brizard, CP, Mee, RB, et al. Arterial switch with full-flow cardiopulmonary bypass and limited circulatory arrest: neurodevelopmental outcome. J Thorac Cardiovasc Surg 2004; 127: 213222.Google Scholar
133. Charpie, JR, Dekeon, MK, Goldberg, CS, Mosca, RS, Bove, EL, Kulik, TJ. Serial blood lactate measurements predict early outcome after neonatal repair or palliation for complex congenital heart disease. J Thorac Cardiovasc Surg 2000; 120: 7380.Google Scholar
134. Munoz, R, Laussen, PC, Palacio, G, Zienko, L, Piercey, G, Wessel, DL. Changes in whole blood lactate levels during cardiopulmonary bypass for surgery for congenital cardiac disease: an early indicator of morbidity and mortality. J Thorac Cardiovasc Surg 2000; 119: 155162.Google Scholar
135. Hoffman, TM, Wernovsky, G, Atz, AM, Kulik, TJ, Nelson, DP, Chang, AC, et al. Efficacy and safety of milrinone in preventing low cardiac output syndrome in infants and children after corrective surgery for congenital heart disease. Circulation 2003; 107: 9961002.Google Scholar
136. Bojan, M, Vicca, S, Boulat, C, Gioanni, S, Pouard, P. Aprotinin, transfusions, and kidney injury in neonates and infants undergoing cardiac surgery. Br J Anaesth 2012; 108: 830837.Google Scholar
137. Pasquali, SK, Li, JS, He, X, Jacobs, ML, O’brien, SM, Hall, M, et al. Comparative analysis of antifibrinolytic medications in pediatric heart surgery. J Thorac Cardiovasc Surg 2012; 143: 550557.Google Scholar
138. Kirklin, JW, Blackstone, EH, Tchervenkov, CI, Castaneda, AR. Clinical outcomes after the arterial switch operation for transposition. Patient, support, procedural, and institutional risk factors. Congenital Heart Surgeons Society. Circulation 1992; 86: 15011515.Google Scholar
139. Prêtre, R, Tamisier, D, Bonhoeffer, P, Mauriat, P, Pouard, P, Sidi, D, et al. Results of the arterial switch operation in neonates with transposed great arteries. Lancet 2001; 357: 18261830.Google Scholar
140. Karamlou, T, McCrindle, BW, Blackstone, EH, Cai, S, Jonas, RA, Bradley, SM, et al. Lesion-specific outcomes in neonates undergoing congenital heart surgery are related predominantly to patient and management factors rather than institution or surgeon experience: a Congenital Heart Surgeons Society Study. J Thorac Cardiovasc Surg 2010; 139: 56977.e1.Google Scholar
141. Khairy, P, Clair, M, Fernandes, SM, Blume, ED, Powell, AJ, Newburger, JW, et al. Cardiovascular outcomes after the arterial switch operation for D-transposition of the great arteries. Circulation 2013; 127: 331339.Google Scholar
142. Metton, O, Calvaruso, D, Gaudin, R, Mussa, S, Raisky, O, Bonnet, D, et al. Intramural coronary arteries and outcome of neonatal arterial switch operation. Eur J Cardiothorac Surg 2010; 37: 12461253.Google Scholar
143. Pasquali, SK, Hasselblad, V, Li, JS, Kong, DF, Sanders, SP. Coronary artery pattern and outcome of arterial switch operation for transposition of the great arteries: a meta-analysis. Circulation 2002; 106: 25752580.Google Scholar
144. Thrupp, SF, Gentles, TL, Kerr, AR, Finucane, K. Arterial switch operation: early and late outcome for intramural coronary arteries. Ann Thorac Surg 2012; 94: 20842090.Google Scholar
145. Tobler, D, Williams, WG, Jegatheeswaran, A, Van Arsdell, GS, McCrindle, BW, Greutmann, M, et al. Cardiac outcomes in young adult survivors of the arterial switch operation for transposition of the great arteries. J Am Coll Cardiol 2010; 56: 5864.Google Scholar
146. Wong, SH, Finucane, K, Kerr, AR, O’donnell, C, West, T, Gentles, TL. Cardiac outcome up to 15 years after the arterial switch operation. Heart Lung Circ 2008; 17: 4853.Google Scholar
147. Bove, EL. Current technique of the arterial switch procedure for transposition of the great arteries. J Card Surg 1989; 4: 193199.Google Scholar
148. Villafane, J, Lantin-Hermoso, MR, Bhatt, AB, Tweddell, JS, Geva, T, Nathan, M, et al. D-transposition of the great arteries: the current era of the arterial switch operation. J Am Coll Cardiol 2014; 64: 498511.Google Scholar
149. Parry, AJ, Thurm, M, Hanley, FL. The use of ‘pericardial hoods’ for maintaining exact coronary artery geometry in the arterial switch operation with complex coronary anatomy. Eur J Cardiothorac Surg 1999; 15: 159164; discussion 64-5. .Google Scholar
150. Tireli, E, Korkut, AK, Basaran, M. Implantation of the coronary arteries after reconstruction of the neoaorta by using pericardial or pulmonary hood techniques. A significant impact on the outcome of arterial switch operations. J Cardiovasc Surg (Torino) 2003; 44: 173178.Google Scholar
151. Yacoub, MH, Radley-Smith, R. Anatomy of the coronary arteries in transposition of the great arteries and methods for their transfer in anatomical correction. Thorax 1978; 33: 418424.Google Scholar
152. Koshiyama, H, Nagashima, M, Matsumura, G, Hiramatsu, T, Nakanishi, T, Yamazaki, K. Arterial switch operation with and without coronary relocation for intramural coronary arteries. Ann Thorac Surg 2016; 102: 13531359.Google Scholar
153. Qamar, ZA, Goldberg, CS, Devaney, EJ, Bove, EL, Ohye, RG. Current risk factors and outcomes for the arterial switch operation. Ann Thorac Surg 2007; 84: 871878.Google Scholar
154. Planche, C, Bruniaux, J, Lacour-Gayet, F, Kachaner, J, Binet, JP, Sidi, D, et al. Switch operation for transposition of the great arteries in neonates. A study of 120 patients. J Thorac Cardiovasc Surg 1988; 96: 354363.Google Scholar
155. Aubert, J, Pannetier, A, Couvelly, JP, Unal, D, Rouault, F, Delarue, A. Transposition of the great arteries. New technique for anatomical correction. Br Heart J 1978; 40: 204208.Google Scholar
156. Brown, EM, Salmon, AP, Lamb, RK. Arterial switch procedure without coronary relocation: a late complication. J Thorac Cardiovasc Surg 1996; 112: 14061407.Google Scholar
157. Suzuki, T, Hotoda, K, Iwazaki, M, Masuoka, A, Katogi, T. Coronary re-implantation after completion of neo-aortic reconstruction in arterial switch operation: accurate intraoperative assessment for the optimal re-implantation site. Keio J Med 2009; 58: 227233.Google Scholar
158. Takeuchi, S, Katogi, T. New technique for the arterial switch operation in difficult situations. Ann Thorac Surg 1990; 50: 1000, discussion 01.Google Scholar
159. Mee, RBB, The Arterial Switch Operation. Surgery for Congenital Heart Defects. John Wiley & Sons, Ltd; 2006: 471487.CrossRefGoogle Scholar
160. Asou, T, Karl, TR, Pawade, A, Mee, RB. Arterial switch: translocation of the intramural coronary artery. Ann Thorac Surg 1994; 57: 461465.Google Scholar
161. Chen, X, Cui, H, Chen, W, Yang, S, Cui, Y, Xia, Y, et al. Early and mid-term results of the arterial switch operation in patients with intramural coronary artery. Pediatr Cardiol 2015; 36: 8488.Google Scholar
162. Lecompte, Y, Zannini, L, Hazan, E, Jarreau, MM, Bex, JP, Tu, TV, et al. Anatomic correction of transposition of the great arteries. J Thorac Cardiovasc Surg 1981; 82: 629631.Google Scholar
163. Delmo Walter, EM, Miera, O, Nasseri, B, Huebler, M, Alexi-Meskishvili, V, Berger, F, et al. Onset of pulmonary stenosis after arterial switch operation for transposition of great arteries with intact ventricular septum. HSR Proc Intensive Care Cardiovasc Anesth 2011; 3: 177187.Google ScholarPubMed
164. Raja, S, Nayak, S, Kaarne, M. Arterial switch operation for simple transposition: three decades later. Intern J Thorac Cardiovasc Surg 2003; 6: 2.Google Scholar
165. Swartz, MF, Sena, A, Atallah-Yunes, N, Meagher, C, Cholette, JM, Gensini, F, et al. Decreased incidence of supravalvar pulmonary stenosis after arterial switch operation. Circulation 2012; 126: S118S122.Google Scholar
166. Ullmann, MV, Gorenflo, M, Bolenz, C, Sebening, C, Goetze, M, Arnold, R, et al. Late results after extended pulmonary artery reconstruction in the arterial switch operation. Ann Thorac Surg 2006; 81: 22592266.Google Scholar
167. Hutter, PA, Kreb, DL, Mantel, SF, Hitchcock, JF, Meijboom, EJ, Bennink, GB. Twenty-five years’ experience with the arterial switch operation. J Thorac Cardiovasc Surg 2002; 124: 790797.Google Scholar
168. Kawata, H, Kishimoto, H, Iwai, S, Ishimaru, K, Saito, T, Kayatani, F, et al. Long term outcome of arterial switch surgery for transposition of the great arteries: evaluation of the reconstruction of the pulmonary artery. Kyobu Geka 2008; 61: 303309.Google Scholar
169. Raju, V, Burkhart, HM, Durham, LA III., Eidem, BW, Phillips, SD, Li, Z, et al. Reoperation after arterial switch: a 27-year experience. Ann Thorac Surg 2013; 95: 21052112.Google Scholar
170. Karl, TR, Cochrane, A, Brizard, CP. Arterial switch operation. Surgical solutions to complex problems. Tex Heart Inst J 1997; 24: 322333.Google Scholar
171. Lalezari, S, Bruggemans, EF, Blom, NA, Hazekamp, MG. Thirty-year experience with the arterial switch operation. Ann Thorac Surg 2011; 92: 973979.Google Scholar
172. Imoto, Y, Kado, H, Asou, T, Shiokawa, Y, Miyake, Y, Yasuda, H, et al. Postoperative pulmonary stenosis after arterial switch operation, comparison in three methods of pulmonary reconstruction: modified Pacifico, autologous pericardial patch, and equine pericardial patch. Kyobu Geka 1995; 48: 433438; discussion 38-41.Google Scholar
173. Rudra, HS, Mavroudis, C, Backer, CL, Kaushal, S, Russell, H, Stewart, RD, et al. The arterial switch operation: 25-year experience with 258 patients. Ann Thorac Surg 2011; 92: 17421746.Google Scholar
174. Sakurai, H, Maeda, M, Miyahara, K, Nakayama, M, Murayama, H, Hasegawa, H, et al. Mid-term results of the arterial switch operation for transposition of the great arteries: effect of fresh autologous pericardial patch in preventing postoperative pulmonary stenosis. Kyobu Geka 2000; 53: 807812; discussion 13-6.Google Scholar
175. Arnold, DM, Fergusson, DA, Chan, AK, Cook, RJ, Fraser, GA, Lim, W, et al. Avoiding transfusions in children undergoing cardiac surgery: a meta-analysis of randomized trials of aprotinin. Anesth Analg 2006; 102: 731737.Google Scholar
176. Fanconi, S. Pulse oximetry for hypoxemia: a warning to users and manufacturers. Intensive Care Med 1989; 15: 540542.Google Scholar
177. Lazzell, VA, Burrows, FA. Stability of the intraoperative arterial to end-tidal carbon dioxide partial pressure difference in children with congenital heart disease. Can J Anaesth 1991; 38: 859865.Google Scholar
178. Stone, JG, Young, WL, Smith, CR, Solomon, RA, Wald, A, Ostapkovich, N, et al. Do standard monitoring sites reflect true brain temperature when profound hypothermia is rapidly induced and reversed? Anesthesiology 1995; 82: 344351.Google Scholar
179. Gottlieb, EA, Fraser, CD Jr, Andropoulos, DB, Diaz, LK. Bilateral monitoring of cerebral oxygen saturation results in recognition of aortic cannula malposition during pediatric congenital heart surgery. Paediatr Anaesth 2006; 16: 787789.Google Scholar
180. Tortoriello, TA, Stayer, SA, Mott, AR, McKenzie, ED, Fraser, CD, Andropoulos, DB, et al. A noninvasive estimation of mixed venous oxygen saturation using near-infrared spectroscopy by cerebral oximetry in pediatric cardiac surgery patients. Paediatr Anaesth 2005; 15: 495503.Google Scholar
181. Andropoulos, DB, Stayer, SA, Diaz, LK, Ramamoorthy, C. Neurological monitoring for congenital heart surgery. Anesth Analg 2004; 99: 13651375.Google Scholar
182. Codaccioni, JL, Velly, LJ, Moubarik, C, Bruder, NJ, Pisano, PS, Guillet, BA. Sevoflurane preconditioning against focal cerebral ischemia: inhibition of apoptosis in the face of transient improvement of neurological outcome. Anesthesiology 2009; 110: 12711278.Google Scholar
183. Lee, HT, Ota-Setlik, A, Fu, Y, Nasr, SH, Emala, CW. Differential protective effects of volatile anesthetics against renal ischemia-reperfusion injury in vivo. Anesthesiology 2004; 101: 13131324.Google Scholar
184. Kussman, BD, Zurakowski, D, Sullivan, L, McGowan, FX, Davis, PJ, Laussen, PC. Evaluation of plasma fentanyl concentrations in infants during cardiopulmonary bypass with low-volume circuits. J Cardiothorac Vasc Anesth 2005; 19: 316321.Google Scholar
185. Newburger, JW, Jonas, RA, Soul, J, Kussman, BD, Bellinger, DC, Laussen, PC, et al. Randomized trial of hematocrit 25% versus 35% during hypothermic cardiopulmonary bypass in infant heart surgery. J Thorac Cardiovasc Surg 2008; 135: 347354.Google Scholar
186. Wypij, D, Jonas, RA, Bellinger, DC, Del Nido, PJ, Mayer, JE Jr, Bacha, EA, et al. The effect of hematocrit during hypothermic cardiopulmonary bypass in infant heart surgery: results from the combined Boston hematocrit trials. J Thorac Cardiovasc Surg 2008; 135: 355360.Google Scholar
187. Sumpelmann, R, Schurholz, T, Thorns, E, Hausdorfer, J. Acid-base, electrolyte and metabolite concentrations in packed red blood cells for major transfusion in infants. Paediatr Anaesth 2001; 11: 169173.Google Scholar
188. Mou, SS, Giroir, BP, Molitor-Kirsch, EA, Leonard, SR, Nikaidoh, H, Nizzi, F, et al. Fresh whole blood versus reconstituted blood for pump priming in heart surgery in infants. N Engl J Med 2004; 351: 16351644.Google Scholar
189. Pasquali, SK, Hall, M, Li, JS, Peterson, ED, Jaggers, J, Lodge, AJ, et al. Corticosteroids and outcome in children undergoing congenital heart surgery: analysis of the Pediatric Health Information Systems database. Circulation 2010; 122: 21232130.Google Scholar
190. Gessler, P, Hohl, V, Carrel, T, Pfenninger, J, Schmid, ER, Baenziger, O, et al. Administration of steroids in pediatric cardiac surgery: impact on clinical outcome and systemic inflammatory response. Pediatr Cardiol 2005; 26: 595600.Google Scholar
191. Graham, EM, Atz, AM, Butts, RJ, Baker, NL, Zyblewski, SC, Deardorff, RL, et al. Standardized preoperative corticosteroid treatment in neonates undergoing cardiac surgery: results from a randomized trial. J Thorac Cardiovasc Surg 2011; 142: 15231529.Google Scholar
192. Pasquali, SK, Li, JS, He, X, Jacobs, ML, O’brien, SM, Hall, M, et al. Perioperative methylprednisolone and outcome in neonates undergoing heart surgery. Pediatrics 2012; 129: e385e391.Google Scholar
193. Scrascia, G, Rotunno, C, Guida, P, Amorese, L, Polieri, D, Codazzi, D, et al. Perioperative steroids administration in pediatric cardiac surgery: a meta-analysis of randomized controlled trials. Pediatr. Crit Care Med 2014; 15: 435442.Google Scholar
194. Miyaji, K, Hannan, RL, Ojito, J, Jacobs, JP, White, JA, Burke, RP. Heparin-coated cardiopulmonary bypass circuit: clinical effects in pediatric cardiac surgery. J Card Surg 2000; 15: 194198.Google Scholar
195. Boning, A, Scheewe, J, Ivers, T, Friedrich, C, Stieh, J, Freitag, S, et al. Phosphorylcholine or heparin coating for pediatric extracorporeal circulation causes similar biologic effects in neonates and infants. J Thorac Cardiovasc Surg 2004; 127: 14581465.Google Scholar
196. Finley, A, Greenberg, C. Review article: heparin sensitivity and resistance: management during cardiopulmonary bypass. Anesth Analg 2013; 116: 12101222.Google Scholar
197. Hickey, PR, Hansen, DD, Wessel, DL, Lang, P, Jonas, RA, Elixson, EM. Blunting of stress responses in the pulmonary circulation of infants by fentanyl. Anesth Analg 1985; 64: 11371142.Google Scholar
198. Liu, J, Ji, B, Long, C, Li, C, Feng, Z. Comparative effectiveness of methylprednisolone and zero-balance ultrafiltration on inflammatory response after pediatric cardiopulmonary bypass. Artif Organs 2007; 31: 571575.Google Scholar
199. Wypij, D, Newburger, JW, Rappaport, LA, duPlessis, AJ, Jonas, RA, Wernovsky, G, et al. The effect of duration of deep hypothermic circulatory arrest in infant heart surgery on late neurodevelopment: the Boston Circulatory Arrest Trial. J Thorac Cardiovasc Surg 2003; 126: 13971403.Google Scholar
200. Ballweg, JA, Wernovsky, G, Ittenbach, RF, Bernbaum, J, Gerdes, M, Gallagher, PR, et al. Hyperglycemia after infant cardiac surgery does not adversely impact neurodevelopmental outcome. Ann Thorac Surg 2007; 84: 20522058.Google Scholar
201. Heying, R, Wehage, E, Schumacher, K, Tassani, P, Haas, F, Lange, R, et al. Dexamethasone pretreatment provides antiinflammatory and myocardial protection in neonatal arterial switch operation. Ann Thorac Surg 2012; 93: 869876.Google Scholar
202. Schroeder, VA, Pearl, JM, Schwartz, SM, Shanley, TP, Manning, PB, Nelson, DP. Combined steroid treatment for congenital heart surgery improves oxygen delivery and reduces postbypass inflammatory mediator expression. Circulation 2003; 107: 28232828.Google Scholar
203. Bergs, J, Hellings, J, Cleemput, I, Zurel, O, De Troyer, V, Van Hiel, M, et al. Systematic review and meta-analysis of the effect of the World Health Organization surgical safety checklist on postoperative complications. Br J Surg 2014; 101: 150158.Google Scholar
204. Boat, AC, Spaeth, JP. Handoff checklists improve the reliability of patient handoffs in the operating room and postanesthesia care unit. Paediatr Anaesth 2013; 23: 647654.Google Scholar
205. Catchpole, KR, de Leval, MR, McEwan, A, Pigott, N, Elliott, MJ, McQuillan, A, et al. Patient handover from surgery to intensive care: using Formula 1 pit-stop and aviation models to improve safety and quality. Paediatr Anaesth 2007; 17: 470478.Google Scholar
206. Joy, BF, Elliott, E, Hardy, C, Sullivan, C, Backer, CL, Kane, JM. Standardized multidisciplinary protocol improves handover of cardiac surgery patients to the intensive care unit. Pediatr Crit Care Med 2011; 12: 304308.Google Scholar
207. Kaufman, J, Twite, M, Barrett, C, Peyton, C, Koehler, J, Rannie, M, et al. A handoff protocol from the cardiovascular operating room to cardiac ICU is associated with improvements in care beyond the immediate postoperative period. Jt Comm J Qual Patient Saf 2013; 39: 306311.Google Scholar
208. Petrovic, MA, Aboumatar, H, Baumgartner, WA, Ulatowski, JA, Moyer, J, Chang, TY, et al. Pilot implementation of a perioperative protocol to guide operating room-to-intensive care unit patient handoffs. J Cardiothorac Vasc Anesth 2012; 26: 1116.Google Scholar
209. Vergales, J, Addison, N, Vendittelli, A, Nicholson, E, Carver, DJ, Stemland, C, et al. Face-to-Face Handoff: Improving Transfer to the Pediatric Intensive Care Unit After Cardiac Surgery. Am J Med Qual 2015; 30: 119125.Google Scholar
210. Seear, MD, Scarfe, JC, LeBlanc, JG. Predicting major adverse events after cardiac surgery in children. Pediatr Crit Care Med 2008; 9: 606611.Google Scholar
211. Bhutta, AT, Ford, JW, Parker, JG, Prodhan, P, Fontenot, EE, Seib, PM, et al. Noninvasive cerebral oximeter as a surrogate for mixed venous saturation in children. Pediatr Cardiol 2007; 28: 3441.Google Scholar
212. Mittnacht, AJ. Near infrared spectroscopy in children at high risk of low perfusion. Curr Opin Anaesthesiol 2010; 23: 342347.Google Scholar
213. Holmes, CL. Vasoactive drugs in the intensive care unit. Curr Opin Crit Care 2005; 11: 413417.Google Scholar
214. Nissen, SE. Report from the Cardiovascular and Renal Drugs Advisory Committee: US Food and Drug Administration; June 15-16, 2005; Gaithersburg, MD. Circulation 2005; 112: 20432046.Google Scholar
215. Zaritsky, A, Chernow, B. Use of catecholamines in pediatrics. J Pediatr 1984; 105: 341350.Google Scholar
216. Deshpande, S, Wolf, M, Kim, D, Kirshbom, P. Simple transposition of the great arteries. In: Eduardo da Cruz DI, Jaggers James (eds). Pediatric and Congenital Cardiology, Cardiac Surgery and Intensive Care. London, UK: Springer-Verlag London Ltd; 2014: 19191940.Google Scholar
217. Burton, GL, Kaufman, J, Goot, BH, da Cruz, EM. The use of arginine vasopressin in neonates following the Norwood procedure. Cardiol Young 2011; 21: 536544.Google Scholar
218. Decker, JA, McCormack, J, Cohen, MI. Arrhythmia management in patients with a common arterial trunk and d-transposition of the great arteries. Cardiol Young 2012; 22: 748754.Google Scholar
219. Shamszad, P, Moore, RA, Ghanayem, N, Cooper, DS. Intensive care management of neonates with d-transposition of the great arteries and common arterial trunk. Cardiol Young 2012; 22: 755760.Google Scholar
220. Texter, KM, Kertesz, NJ, Friedman, RA, Fenrich, AL Jr. Atrial flutter in infants. J Am Coll Cardiol 2006; 48: 10401046.Google Scholar
221. The Esmolol Research Group. Intravenous esmolol for the treatment of supraventricular tachyarrhythmia: results of a multicenter, baseline-controlled safety and efficacy study in 160 patients. Am Heart J 1986; 112: 498505.Google Scholar
222. Trippel, DL, Wiest, DB, Gillette, PC. Cardiovascular and antiarrhythmic effects of esmolol in children. J Pediatr 1991; 119: 142147.Google Scholar
223. Benson, DW Jr, Dunnigan, A, Green, TP, Benditt, DG, Schneider, SP. Periodic procainamide for paroxysmal tachycardia. Circulation 1985; 72: 147152.Google Scholar
224. Dubin, A. Antiarrhythmic medications. In: CS Ricardo Munoz, Roth Stephen, da Cruz Eduardo (eds). Handbook of Pediatric Cardiovascular Drugs. London, UK: Springer-Verlag London Ltd; 2008: 150189.Google Scholar
225. Mandapati, R, Byrum, CJ, Kavey, RE, Smith, FC, Kveselis, DA, Hannan, WP, et al. Procainamide for rate control of postsurgical junctional tachycardia. Pediatr Cardiol 2000; 21: 123128.Google Scholar
226. Coumel, P, Fidelle, J. Amiodarone in the treatment of cardiac arrhythmias in children: one hundred thirty-five cases. Am Heart J 1980; 100: 10631069.Google Scholar
227. Figa, FH, Gow, RM, Hamilton, RM, Freedom, RM. Clinical efficacy and safety of intravenous Amiodarone in infants and children. Am J Cardiol 1994; 74: 573577.Google Scholar
228. Perry, JC, Fenrich, AL, Hulse, JE, Triedman, JK, Friedman, RA, Lamberti, JJ. Pediatric use of intravenous amiodarone: efficacy and safety in critically ill patients from a multicenter protocol. J Am Coll Cardiol 1996; 27: 12461250.Google Scholar
229. Perry, JC, Knilans, TK, Marlow, D, Denfield, SW, Fenrich, AL, Friedman, RA. Intravenous amiodarone for life-threatening tachyarrhythmias in children and young adults. J Am Coll Cardiol 1993; 22: 9598.Google Scholar
230. Maragnes, P, Tipple, M, Fournier, A. Effectiveness of oral sotalol for treatment of pediatric arrhythmias. Am J Cardiol 1992; 69: 751754.Google Scholar
231. Brugada, J, Blom, N, Sarquella-Brugada, G, Blomstrom-Lundqvist, C, Deanfield, J, Janousek, J. Pharmacological and non-pharmacological therapy for arrhythmias in the pediatric population: EHRA and AEPC-Arrhythmia Working Group joint consensus statement. Europace 2013; 15: 13371382.Google Scholar
232. Vernon, DD, Witte, MK. Effect of neuromuscular blockade on oxygen consumption and energy expenditure in sedated, mechanically ventilated children. Crit Care Med 2000; 28: 15691571.Google Scholar
233. Tibby, SM, Hatherill, M, Marsh, MJ, Murdoch, IA. Clinicians’ abilities to estimate cardiac index in ventilated children and infants. Arch Dis Child 1997; 77: 516518.Google Scholar
234. Bisoi, AK, Ahmed, T, Malankar, DP, Chauhan, S, Das, S, Sharma, P, et al. Midterm outcome of primary arterial switch operation beyond six weeks of life in children with transposition of great arteries and intact ventricular septum. World J Pediatr Congenit Heart Surg 2014; 5: 219225.Google Scholar
235. Ambuel, B, Hamlett, KW, Marx, CM, Blumer, JL. Assessing distress in pediatric intensive care environments: the COMFORT scale. J Pediatr Psychol 1992; 17: 95109.Google Scholar
236. Rawlinson, E, Howard, R. Post-operative sedation and analgesia. In: Eduardo da Cruz DI, Jaggers James (eds). Pediatric and Congenital Cardiology, Cardiac Surgery and Intensive Care. London, UK: Springer-Verlag London Ltd; 2014: 705719.Google Scholar
237. Chrysostomou, C, Morell, VO, Wearden, P, Sanchez-de-Toledo, J, Jooste, EH, Beerman, L. Dexmedetomidine: therapeutic use for the termination of reentrant supraventricular tachycardia. Congenit Heart Dis 2013; 8: 4856.Google Scholar
238. Hosokawa, K, Shime, N, Kato, Y, Taniguchi, A, Maeda, Y, Miyazaki, T, et al. Dexmedetomidine sedation in children after cardiac surgery. Pediatr Crit Care Med 2010; 11: 3943.Google Scholar
239. Parent, BA, Munoz, R, Shiderly, D, Chrysostomou, C. Use of dexmedetomidine in sustained ventricular tachycardia. Anaesth Intensive Care 2010; 38: 781.Google Scholar
240. Tobias, JD, Chrysostomou, C. Dexmedetomidine: antiarrhythmic effects in the pediatric cardiac patient. Pediatr Cardiol 2013; 34: 779785.Google Scholar
241. Martin, LD, Bratton, SL, Quint, P, Mayock, DE. Prospective documentation of sedative, analgesic, and neuromuscular blocking agent use in infants and children in the intensive care unit: a multicenter perspective. Pediatr Crit Care Med 2001; 2: 205210.Google Scholar
242. Tabarki, B, Coffinieres, A, Van Den Bergh, P, Huault, G, Landrieu, P, Sebire, G. Critical illness neuromuscular disease: clinical, electrophysiological, and prognostic aspects. Arch Dis Child 2002; 86: 103107.Google Scholar
243. Foglia, E, Meier, MD, Elward, A. Ventilator-associated pneumonia in neonatal and pediatric intensive care unit patients. Clin Microbiol Rev 2007; 20: 409425.Google Scholar
244. Rosenthal, VD, Bijie, H, Maki, DG, Mehta, Y, Apisarnthanarak, A, Medeiros, EA, et al. International Nosocomial Infection Control Consortium (INICC) report, data summary of 36 countries, for 2004-2009. Am J Infect Control 2012; 40: 396407.Google Scholar
245. Elward, AM. Pediatric ventilator-associated pneumonia. Pediatr Infect Dis 2003; 22: 445446.Google Scholar
246. Elward, AM, Warren, DK, Fraser, VJ. Ventilator-associated pneumonia in pediatric intensive care unit patients: risk factors and outcomes. Pediatrics 2002; 109: 758764.Google Scholar
247. Gil-Ruiz Gil-Esparza, MA, Alcaraz Romero, AJ, Romero Otero, A, Gil Villanueva, N, Sanavia Moran, E, Rodriguez Sanchez de la Blanca, A, et al. Prognostic relevance of early AKI according to pRIFLE criteria in children undergoing cardiac surgery. Pediatr Nephrol 2014; 29: 12651272.Google Scholar
248. Lex, DJ, Toth, R, Cserep, Z, Alexander, SI, Breuer, T, Sapi, E, et al. A comparison of the systems for the identification of postoperative acute kidney injury in pediatric cardiac patients. Ann Thorac Surg 2014; 97: 202210.Google Scholar
249. Ricci, Z, Di Nardo, M, Iacoella, C, Netto, R, Picca, S, Cogo, P. Pediatric RIFLE for acute kidney injury diagnosis and prognosis for children undergoing cardiac surgery: a single-center prospective observational study. Pediatr Cardiol 2013; 34: 14041408.Google Scholar
250. Toth, R, Breuer, T, Cserep, Z, Lex, D, Fazekas, L, Sapi, E, et al. Acute kidney injury is associated with higher morbidity and resource utilization in pediatric patients undergoing heart surgery. Ann Thorac Surg 2012; 93: 19841990.Google Scholar
251. Watkins, SC, Williamson, K, Davidson, M, Donahue, BS. Long-term mortality associated with acute kidney injury in children following congenital cardiac surgery. Paediatr Anaesth 2014; 24: 919926.Google Scholar
252. da Cruz, E, Rimensberger, P. Inotropic and vasoactive drugs. In: Ricardo Munoz CS, Roth Stephen, da Cruz Eduardo (eds). Handbook of Pediatric Cardiovascular Drugs. London, UK: Springer-Verlag London Ltd; 2008: 3376.Google Scholar
253. Roth, S, Munoz, R, Schmitt, C, da Cruz, E, Kaufman, J, Tissot, C. Vasodilators. In: Ricardo Munoz CS, Roth Stephen, Cruz Eduardo da (eds). Handbook of Pediatric Cardiovascular Drugs. London, UK: Springer-Verlag London Ltd; 2008: 77118.Google Scholar
254. Moons, P, Gewillig, M, Sluysmans, T, Verhaaren, H, Viart, P, Massin, M, et al. Long term outcome up to 30 years after the Mustard or Senning operation: a nationwide multicentre study in Belgium. Heart 2004; 90: 307313.Google Scholar
255. Murphy, DJ Jr. Transposition of the great arteries: long-term outcome and current management. Curr Cardiol Rep 2005; 7: 299304.Google Scholar
256. Puley, G, Siu, S, Connelly, M, Harrison, D, Webb, G, Williams, WG, et al. Arrhythmia and survival in patients >18 years of age after the mustard procedure for complete transposition of the great arteries. Am J Cardiol 1999; 83: 10801084.Google Scholar
257. Roos-Hesselink, JW, Meijboom, FJ, Spitaels, SE, van Domburg, R, van Rijen, EH, Utens, EM, et al. Decline in ventricular function and clinical condition after Mustard repair for transposition of the great arteries (a prospective study of 22-29 years). Eur Heart J 2004; 25: 12641270.Google Scholar
258. Wilson, NJ, Clarkson, PM, Barratt-Boyes, BG, Calder, AL, Whitlock, RM, Easthope, RN, et al. Long-term outcome after the mustard repair for simple transposition of the great arteries. 28-year follow-up. J Am Coll Cardiol 1998; 32: 758765.Google Scholar
259. Birnie, D, Tometzki, A, Curzio, J, Houston, A, Hood, S, Swan, L, et al. Outcomes of transposition of the great arteries in the ear of atrial inflow correction. Heart 1998; 80: 170173.Google Scholar
260. Sun, ZH, Happonen, JM, Bennhagen, R, Sairanen, H, Pesonen, E, Toivonen, L, et al. Increased QT dispersion and loss of sinus rhythm as risk factors for late sudden death after Mustard or Senning procedures for transposition of the great arteries. Am J Cardiol 2004; 94: 138141.Google Scholar
261. Gewillig, M, Cullen, S, Mertens, B, Lesaffre, E, Deanfield, J. Risk factors for arrhythmia and death after Mustard operation for simple transposition of the great arteries. Circulation 1991; 84: 187192.Google Scholar
262. Duncan, BW, Mee, RB. Management of the failing systemic right ventricle. Semin Thorac Cardiovasc Surg 2005; 17: 160169.Google Scholar
263. Barron, DJ, Jones, TJ, Brawn, WJ. The Senning procedure as part of the double-switch operations for congenitally corrected transposition of the great arteries. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2011; 14: 109115.Google Scholar
264. Mee, RB. The double switch operation with accent on the Senning component. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2005; 8: 5765.Google Scholar
265. Senning, A. Surgical correction of transposition of the great vessels. Surgery 1959; 45: 966980.Google Scholar
266. Mustard, WT. Successful two-stage correction of transposition of the great vessels. Surgery 1964; 55: 469472.Google Scholar
267. Winter, MM, Bouma, BJ, Groenink, M, Konings, TC, Tijssen, JG, van Veldhuisen, DJ, et al. Latest insights in therapeutic options for systemic right ventricular failure: a comparison with left ventricular failure. Heart 2009; 95: 960963.Google Scholar
268. Hechter, SJ, Fredriksen, PM, Liu, P, Veldtman, G, Merchant, N, Freeman, M, et al. Angiotensin-converting enzyme inhibitors in adults after the Mustard procedure. Am J Cardiol 2001; 87: 660663.Google Scholar
269. Gelatt, M, Hamilton, RM, McCrindle, BW, Connelly, M, Davis, A, Harris, L, et al. Arrhythmia and mortality after the Mustard procedure: a 30-year single-center experience. J Am Coll Cardiol 1997; 29: 194201.Google Scholar
270. Diller, GP, Okonko, D, Uebing, A, Ho, SY, Gatzoulis, MA. Cardiac resynchronization therapy for adult congenital heart disease patients with a systemic right ventricle: analysis of feasibility and review of early experience. Europace 2006; 8: 267272.Google Scholar
271. Janousek, J, Tomek, V, Chaloupecky, VA, Reich, O, Gebauer, RA, Kautzner, J, et al. Cardiac resynchronization therapy: a novel adjunct to the treatment and prevention of systemic right ventricular failure. J Am Coll Cardiol 2004; 44: 19271931.Google Scholar
272. Triedman, JK. Arrhythmias in adults with congenital heart disease. Heart 2002; 87: 383389.Google Scholar
273. Kenleigh, D, Edens, RE, Bates, MJ, Turek, JW. Use of heart ware ventricular assist system for systemic ventricular support of a pediatric patient after Mustard procedure. World J Pediatr Congenit Heart Surg 2015; 6: 339341.Google Scholar
274. Warnes, CA. Transposition of the great arteries. Circulation 2006; 114: 26992709.Google Scholar
275. van Son, JA, Danielson, GK, Huhta, JC, Warnes, CA, Edwards, WD, Schaff, HV, et al. Late results of systemic atrioventricular valve replacement in corrected transposition. J Thorac Cardiovasc Surg 1995; 109: 642652.Google Scholar
276. Scherptong, RW, Vliegen, HW, Winter, MM, Holman, ER, Mulder, BJ, van der Wall, EE, et al. Tricuspid valve surgery in adults with a dysfunctional systemic right ventricle: repair or replace? Circulation 2009; 119: 14671472.Google Scholar
277. Mavroudis, C, Backer, CL. Arterial switch after failed atrial baffle procedures for transposition of the great arteries. Ann Thorac Surg 2000; 69: 851857.Google Scholar
278. Poirier, NC, Yu, JH, Brizard, CP, Mee, RB. Long-term results of left ventricular reconditioning and anatomic correction for systemic right ventricular dysfunction after atrial switch procedures. J Thorac Cardiovasc Surg 2004; 127: 975981.Google Scholar
279. Corno, AF. FloWatch device for adjustable pulmonary artery banding. J Thorac Cardiovasc Surg 2013; 145: 1144.Google Scholar
280. Dibardino, DJ, Kleeman, K, Bove, EL. A method of transcutaneously adjustable pulmonary artery banding for staged left ventricular retraining. J Thorac Cardiovasc Surg 2012; 144: 553556.Google Scholar
281. Padalino, MA, Stellin, G, Brawn, WJ, Fasoli, G, Daliento, L, Milanesi, O, et al. Arterial switch operation after left ventricular retraining in the adult. Ann Thorac Surg 2000; 70: 17531757.Google Scholar
282. Daebritz, SH, Tiete, AR, Sachweh, JS, Engelhardt, W, von Bernuth, G, Messmer, BJ. Systemic right ventricular failure after atrial switch operation: midterm results of conversion into an arterial switch. Ann Thorac Surg 2001; 71: 12551259.Google Scholar
283. Benzaquen, BS, Webb, GD, Colman, JM, Therrien, J. Arterial switch operation after Mustard procedures in adult patients with transposition of the great arteries: is it time to revise our strategy? Am Heart J 2004; 147: E8.Google Scholar
284. Mee, RB. Severe right ventricular failure after Mustard or Senning operation. Two-stage repair: pulmonary artery banding and switch. J Thorac Cardiovasc Surg 1986; 92: 385390.Google Scholar
285. Chang, AC, Wernovsky, G, Wessel, DL, Freed, MD, Parness, IA, Perry, SB, et al. Surgical management of late right ventricular failure after Mustard or Senning repair. Circulation 1992; 86: 140149.Google ScholarPubMed
286. van Son, JA, Reddy, VM, Silverman, NH, Hanley, FL. Regression of tricuspid regurgitation after two-stage arterial switch operation for failing systemic ventricle after atrial inversion operation. J Thorac Cardiovasc Surg 1996; 111: 342347.Google Scholar
287. Hetzer, R, Weng, Y, Delmo Walter, EM. State of the art in paediatric heart transplantation: the Berlin experience. Eur J Cardiothorac Surg 2013; 43: 258267.Google Scholar
288. Canter, CE, Shaddy, RE, Bernstein, D, Hsu, DT, Chrisant, MR, Kirklin, JK, et al. Indications for heart transplantation in pediatric heart disease: a scientific statement from the American Heart Association Council on Cardiovascular Disease in the Young; the Councils on Clinical Cardiology, Cardiovascular Nursing, and Cardiovascular Surgery and Anesthesia; and the Quality of Care and Outcomes Research Interdisciplinary Working Group. Circulation 2007; 115: 658676.Google Scholar
289. Kim, SJ, Kim, WH, Lim, C, Oh, SS, Kim, YM. Commissural malalignment of aortic-pulmonary sinus in complete transposition of great arteries. Ann Thorac Surg 2003; 76: 19061910.Google Scholar
290. Konstantinov, IE, Fricke, TA, D’Udekem, Y, Radford, DJ. Translocation of a single coronary artery from the nonfacing sinus in the arterial switch operation: long-term patency of the interposition graft. J Thorac Cardiovasc Surg 2010; 140: 11931194.Google Scholar
291. Talwar, S, Nair, VV, Choudhary, SK, Airan, B. Atrial switch operation in the current era: modifications and pitfalls. World J Pediatr Congenit Heart Surg 2012; 3: 96103.Google Scholar
292. Wernovsky, G, Rome, JJ, Tabbutt, S, Rychik, J, Cohen, MS, Paridon, SM, et al. Guidelines for the outpatient management of complex congenital heart disease. Congenit Heart Dis 2006; 1: 1026.Google Scholar
293. Angeli, E, Raisky, O, Bonnet, D, Sidi, D, Vouhé, PR. Late reoperations after neonatal arterial switch operation for transposition of the great arteries. Eur J Cardiothorac Surg 2008; 34: 3236.Google Scholar
294. Valsangiacomo Buechel, ER, Grosse-Wortmann, L, Fratz, S, Eichhorn, J, Sarikouch, S, Greil, GF, et al. Indications for cardiovascular magnetic resonance in children with congenital and acquired heart disease: an expert consensus paper of the Imaging Working Group of the AEPC and the Cardiovascular Magnetic Resonance Section of the EACVI. Eur Heart J Cardiovasc Imaging 2015; 16: 281297.Google Scholar
295. Cohen, MS, Eidem, BW, Cetta, F, Fogel, MA, Frommelt, PC, Ganame, J, et al. Multimodality imaging guidelines of patients with transposition of the great arteries: a report from the American Society of Echocardiography developed in collaboration with the Society for Cardiovascular Magnetic Resonance and the Society of Cardiovascular Computed Tomography. J Am Soc Echocardiogr 2016; 29: 571621.Google Scholar
296. Fratz, S, Chung, T, Greil, GF, Samyn, MM, Taylor, AM, Valsangiacomo Buechel, ER, et al. Guidelines and protocols for cardiovascular magnetic resonance in children and adults with congenital heart disease: SCMR expert consensus group on congenital heart disease. J Cardiovasc Magn Reson 2013; 15: 51.Google Scholar
297. Legendre, A, Losay, J, Touchot-Kone, A, Serraf, A, Belli, E, Piot, JD, et al. Coronary events after arterial switch operation for transposition of the great arteries. Circulation 2003; 108 (Suppl 1): 186190.Google Scholar
298. Ou, P, Khraiche, D, Celermajer, DS, Agnoletti, G, Le Quan Sang, KH, Thalabard, JC, et al. Mechanisms of coronary complications after the arterial switch for transposition of the great arteries. J Thorac Cardiovasc Surg 2013; 145: 12631269.Google Scholar
299. Cho, I, Chang, HJ, Shin, S, Sung, JM, Lin, FY, et al. Incremental prognostic utility of coronary CT angiography for asymptomatic patients based upon extent and severity of coronary artery calcium: results from the COronary CT Angiography EvaluatioN For Clinical Outcomes InteRnational Multicenter (CONFIRM) study. Eur Heart J 2015; 36: 501508.Google Scholar
300. Ou, P, Celermajer, DS, Marini, D, Agnoletti, G, Vouhé, P, Brunelle, F, et al. Safety and accuracy of 64-slice computed tomography coronary angiography in children after the arterial switch operation for transposition of the great arteries. JACC Cardiovasc Imaging 2008; 1: 331339.Google Scholar
301. Tobler, D, Motwani, M, Wald, RM, Roche, SL, Verocai, F, Iwanochko, RM, et al. Evaluation of a comprehensive cardiovascular magnetic resonance protocol in young adults late after the arterial switch operation for d-transposition of the great arteries. J Cardiovasc Magn Reson 2014; 16: 98.Google Scholar
302. Buechel, ER, Balmer, C, Bauersfeld, U, Kellenberger, CJ, Schwitter, J. Feasibility of perfusion cardiovascular magnetic resonance in paediatric patients. J Cardiovasc Magn Reson 2009; 11: 51.Google Scholar
303. Taylor, AM, Dymarkowski, S, Hamaekers, P, Razavi, R, Gewillig, M, Mertens, L, et al. MR coronary angiography and late-enhancement myocardial MR in children who underwent arterial switch surgery for transposition of great arteries. Radiology 2005; 234: 542547.Google Scholar
304. Day, RW, Laks, H, Drinkwater, DC. The influence of coronary anatomy on the arterial switch operation in neonates. J Thorac Cardiovasc Surg 1992; 104: 706712.Google Scholar
305. Yamaguchi, M, Hosokawa, Y, Imai, Y, Kurosawa, H, Yasui, H, Yagihara, T, et al. Early and midterm results of the arterial switch operation for transposition of the great arteries in Japan. J Thorac Cardiovasc Surg 1990; 100: 261269.Google Scholar
306. el-Said, G, Rosenberg, HS, Mullins, CE, Hallman, GL, Cooley, DA, McNamara, DG. Dysrhythmias after Mustard’s operation for transposition of the treat arteries. Am J Cardiol 1972; 30: 526532.Google Scholar
307. Isaacson, R, Titus, JL, Merideth, J, Feldt, RH, McGoon, DC. Apparent interruption of atrial conduction pathways after surgical repair of transposition of great arteries. Am J Cardiol 1972; 30: 533535.Google Scholar
308. Martin, TC, Smith, L, Hernandez, A, Weldon, CS. Dysrhythmias following the Senning operation for dextro-transposition of the great arteries. J Thorac Cardiovasc Surg 1983; 85: 928932.Google Scholar
309. Hayashi, G, Kurosaki, K, Echigo, S, Kado, H, Fukushima, N, Yokota, M, et al. Prevalence of arrhythmias and their risk factors mid- and long-term after the arterial switch operation. Pediatr Cardiol 2006; 27: 689694.Google Scholar
310. Yamazaki, A, Yamamoto, N, Sakamoto, T, Ishihara, K, Iwata, Y, Matsumura, G, et al. Long-term outcomes and social independence level after arterial switch operation. Eur J Cardiothorac Surg 2008; 33: 239243.Google Scholar
311. Rhodes, LA, Wernovsky, G, Keane, JF, Mayer, JE Jr, Shuren, A, Dindy, C, et al. Arrhythmias and intracardiac conduction after the arterial switch operation. J Thorac Cardiovasc Surg 1995; 109: 303310.Google Scholar
312. Delaney, JW, Moltedo, JM, Dziura, JD, Kopf, GS, Snyder, CS. Early postoperative arrhythmias after pediatric cardiac surgery. J Thorac Cardiovasc Surg 2006; 131: 12961300.Google Scholar
313. Losay, J, Touchot, A, Serraf, A, Litvinova, A, Lambert, V, Piot, JD, et al. Late outcome after arterial switch operation for transposition of the great arteries. Circulation 2001; 104: 121126.Google Scholar
314. Horer, J, Schreiber, C, Cleuziou, J, Vogt, M, Prodan, Z, Busch, R, et al. Improvement in long-term survival after hospital discharge but not in freedom from reoperation after the change from atrial to arterial switch for transposition of the great arteries. J Thorac Cardiovasc Surg 2009; 137: 347354.Google Scholar
315. Rodriguez Puras, MJ, Cabeza-Letran, L, Romero-Vazquianez, M, Santos de Soto, J, Hosseinpour, R, Gil Fournier, M, et al. Mid-term morbidity and mortality of patients after arterial switch operation in infancy for transposition of the great arteries. Rev Esp Cardiol (Engl Ed) 2014; 67: 181188.Google Scholar
316. Haas, F, Wottke, M, Poppert, H, Meisner, H. Long-term survival and functional follow-up in patients after the arterial switch operation. Ann Thorac Surg 1999; 68: 16921697.Google Scholar
317. Freed, DH, Robertson, CM, Sauve, RS, Joffe, AR, Rebeyka, IM, Ross, DB, et al. Intermediate-term outcomes of the arterial switch operation for transposition of great arteries in neonates: alive but well? J Thorac Cardiovasc Surg 2006; 132: 845852.Google Scholar
318. Nogi, S, McCrindle, BW, Boutin, C, Williams, WG, Freedom, RM, Benson, LN. Fate of the neopulmonary valve after the arterial switch operation in neonates. J Thorac Cardiovasc Surg 1998; 115: 557562.Google Scholar
319. Wernovsky, G, Mayer, JE Jr, Jonas, RA, Hanley, FL, Blackstone, EH, Kirklin, JW, et al. Factors influencing early and late outcome of the arterial switch operation for transposition of the great arteries. J Thorac Cardiovasc Surg 1995; 109: 289301.Google Scholar
320. Williams, WG, Quaegebeur, JM, Kirklin, JW, Blackstone, EH. Outflow obstruction after the arterial switch operation: a multiinstitutional study. Congenital Heart Surgeons Society. J Thorac Cardiovasc Surg 1997; 114: 975987.Google Scholar
321. Vargo, P, Mavroudis, C, Stewart, RD, Backer, CL. Late complications following the arterial switch operation. World J Pediatr Congenit Heart Surg 2011; 2: 3742.Google Scholar
322. Bove, T, De Meulder, F, Vandenplas, G, De Groote, K, Panzer, J, Suys, B, et al. Midterm assessment of the reconstructed arteries after the arterial switch operation. Ann Thorac Surg 2008; 85: 823830.Google Scholar
323. Prifti, E, Crucean, A, Bonacchi, M, Bernabei, M, Murzi, B, Luisi, SV, et al. Early and long term outcome of the arterial switch operation for transposition of the great arteries: predictors and functional evaluation. Eur J Cardiothorac Surg 2002; 22: 864873.Google Scholar
324. Lupinetti, FM, Bove, EL, Minich, LL, Snider, AR, Callow, LB, Meliones, JN, et al. Intermediate-term survival and functional results after arterial repair for transposition of the great arteries. J Thorac Cardiovasc Surg 1992; 103: 421427.Google Scholar
325. Paillole, C, Sidi, D, Kachaner, J, Planche, C, Belot, JP, Villain, E, et al. Fate of pulmonary artery after anatomic correction of simple transposition of great arteries in newborn infants. Circulation 1988; 78: 870876.Google Scholar
326. Wernovsky, G, Hougen, TJ, Walsh, EP, Sholler, GF, Colan, SD, Sanders, SP, et al. Midterm results after the arterial switch operation for transposition of the great arteries with intact ventricular septum: clinical, hemodynamic, echocardiographic, and electrophysiologic data. Circulation 1988; 77: 13331344.Google Scholar
327. Spiegelenberg, SR, Hutter, PA, van de Wal, HJ, Hitchcock, JF, Meijboom, EJ, Harinck, E. Late re-interventions following arterial switch operations in transposition of the great arteries. Incidence and surgical treatment of postoperative pulmonary stenosis. Eur J Cardiothorac Surg 1995; 9: 710.Google Scholar
328. Zeevi, B, Keane, JF, Perry, SB, Lock, JE. Balloon dilation of postoperative right ventricular outflow obstructions. J Am Coll Cardiol 1989; 14: 401408.Google Scholar
329. Kuroczynski, W, Kampmann, C, Choi, YH, Hilker, M, Wippermann, F, David, M, et al. Treatment of supravalvular pulmonary stenosis after arterial switch operations (ASO). Z Kardiol 2001; 90: 498502.Google Scholar
330. Massin, MM, Nitsch, GB, Dabritz, S, Seghaye, MC, Messmer, BJ, von Bernuth, G. Growth of pulmonary artery after arterial switch operation for simple transposition of the great arteries. Eur J Pediatr 1998; 157: 95100.Google Scholar
331. Nakanishi, T, Momoi, N, Satoh, M, Yamada, M, Terada, M, Nakazawa, M, et al. Growth of the neopulmonary valve annulus after arterial switch operation in transposition of the great arteries. Circulation 1996; 94: 2731.Google Scholar
332. Serraf, A, Bruniaux, J, Lacour-Gayet, F, Sidi, D, Kachaner, J, Bouchart, F, et al. Anatomic correction of transposition of the great arteries with ventricular septal defect. Experience with 118 cases. J Thorac Cardiovasc Surg 1991; 102: 140147.Google Scholar
333. Raja, SG, Shauq, A, Kaarne, M. Outcomes after arterial switch operation for simple transposition. Asian Cardiovasc Thorac Ann 2005; 13: 190198.Google Scholar
334. Santoro, G, Di Carlo, D, Formigari, R, Ballerini, L. Late onset pulmonary valvar stenosis after arterial switch operation for transposition of the great arteries. Heart 1998; 79: 311312.Google Scholar
335. Formigari, R, Santoro, G, Guccione, P, Giamberti, A, Pasquini, L, Grigioni, M, et al. Treatment of pulmonary artery stenosis after arterial switch operation: stent implantation vs. balloon angioplasty. Catheter Cardiovasc Interv 2000; 50: 207211.Google Scholar
336. Tzifa, A, Papagiannis, J, Qureshi, S. Iatrogenic aortopulmonary window after balloon dilation of left pulmonary artery stenosis following arterial switch operation. J Invasive Cardiol 2013; 25: E188E190.Google Scholar
337. Torres, A, Sanders, SP, Vincent, JA, El-Said, HG, Leahy, RA, Padera, RF, et al. Iatrogenic aortopulmonary communications after transcatheter interventions on the right ventricular outflow tract or pulmonary artery: pathophysiologic, diagnostic, and management considerations. Catheter Cardiovasc Interv 2015; 86: 438452.Google Scholar
338. Gleason, MM, Chin, AJ, Andrews, BA, Barber, G, Helton, JG, Murphy, JD, et al. Two-dimensional and Doppler echocardiographic assessment of neonatal arterial repair for transposition of the great arteries. J Am Coll Cardiol 1989; 13: 13201328.Google Scholar
339. Martin, MM, Snider, AR, Bove, EL, Serwer, GA, Rosenthal, A, Peters, J, et al. Two-dimensional and Doppler echocardiographic evaluation after arterial switch repair in infancy for complete transposition of the great arteries. Am J Cardiol 1989; 63: 332336.Google Scholar
340. Hovels-Gurich, HH, Seghaye, MC, Ma, Q, Miskova, M, Minkenberg, R, Messmer, BJ, et al. Long-term results of cardiac and general health status in children after neonatal arterial switch operation. Ann Thorac Surg 2003; 75: 935943.Google Scholar
341. Geva, T. Indications for pulmonary valve replacement in repaired tetralogy of Fallot: the quest continues. Circulation 2013; 128: 18551857.Google Scholar
342. Eicken, A, Ewert, P, Hager, A, Peters, B, Fratz, S, Kuehne, T, et al. Percutaneous pulmonary valve implantation: two-centre experience with more than 100 patients. Eur Heart J 2011; 32: 12601265.Google Scholar
343. Thanopoulos, BV, Giannakoulas, G, Arampatzis, CA. Percutaneous pulmonary valve implantation in the native right ventricular outflow tract. Catheter Cardiovasc Interv 2012; 79: 427429.Google Scholar
344. Biermann, D, Schonebeck, J, Rebel, M, Weil, J, Dodge-Khatami, A. Left coronary artery occlusion after percutaneous pulmonary valve implantation. Ann Thorac Surg 2012; 94: E7E9.Google Scholar
345. Delmo Walter, EM, Huebler, M, Alexi-Meshkishvili, V, Sill, B, Berger, F, Hetzer, R. Fate of the aortic valve following the arterial switch operation. J Card Surg 2010; 25: 730736.Google Scholar
346. Marino, BS, Wernovsky, G, McElhinney, DB, Jawad, A, Kreb, DL, Mantel, SF, et al. Neo-aortic valvar function after the arterial switch. Cardiol Young 2006; 16: 481489.Google Scholar
347. McMahon, CJ, Ravekes, WJ, Smith, EO, Denfield, SW, Pignatelli, RH, Altman, CA, et al. Risk factors for neo-aortic root enlargement and aortic regurgitation following arterial switch operation. Pediatr Cardiol 2004; 25: 329335.Google Scholar
348. Schwartz, ML, Gauvreau, K, del Nido, P, Mayer, JE, Colan, SD. Long-term predictors of aortic root dilation and aortic regurgitation after arterial switch operation. Circulation 2004; 110: 128132.Google Scholar
349. Martin, RP, Ettedgui, JA, Qureshi, SA, Gibbs, JL, Baker, EJ, Radley-Smith, R, et al. A quantitative evaluation of aortic regurgitation after anatomic correction of transposition of the great arteries. J Am Coll Cardiol 1988; 12: 12811284.Google Scholar
350. Lange, R, Cleuziou, J, Horer, J, Holper, K, Vogt, M, Tassani-Prell, P, et al. Risk factors for aortic insufficiency and aortic valve replacement after the arterial switch operation. Eur J Cardiothorac Surg 2008; 34: 711717.Google Scholar
351. Daebritz, SH, Nollert, G, Sachweh, JS, Engelhardt, W, von Bernuth, G, Messmer, BJ. Anatomical risk factors for mortality and cardiac morbidity after arterial switch operation. Ann Thorac Surg 2000; 69: 18801886.Google Scholar
352. Hwang, HY, Kim, WH, Kwak, JG, Lee, JR, Kim, YJ, Rho, JR, et al. Mid-term follow-up of neoaortic regurgitation after the arterial switch operation for transposition of the great arteries. Eur J Cardiothorac Surg 2006; 29: 162167.Google Scholar
353. Hutter, PA, Thomeer, BJ, Jansen, P, Hitchcock, JF, Faber, JA, Meijboom, EJ, et al. Fate of the aortic root after arterial switch operation. Eur J Cardiothorac Surg 2001; 20: 8288.Google Scholar
354. Mavroudis, C, Stewart, RD, Backer, CL, Rudra, H, Vargo, P, Jacobs, ML. Reoperative techniques for complications after arterial switch. Ann Thorac Surg 2011; 92: 17471754.Google Scholar
355. Losay, J, Touchot, A, Capderou, A, Piot, JD, Belli, E, Planche, C, et al. Aortic valve regurgitation after arterial switch operation for transposition of the great arteries: incidence, risk factors, and outcome. J Am Coll Cardiol 2006; 47: 20572062.Google Scholar
356. del Nido, PJ, Schwartz, ML. Aortic regurgitation after arterial switch operation. J Am Coll Cardiol 2006; 47: 20632064.Google Scholar
357. Jenkins, KJ, Hanley, FL, Colan, SD, Mayer, JE Jr, Castaneda, AR, Wernovsky, G. Function of the anatomic pulmonary valve in the systemic circulation. Circulation 1991; 84: 173179.Google Scholar
358. Lalezari, S, Hazekamp, MG, Bartelings, MM, Schoof, PH, Gittenberger-De Groot, AC. Pulmonary artery remodeling in transposition of the great arteries: relevance for neoaortic root dilatation. J Thorac Cardiovasc Surg 2003; 126: 10531060.Google Scholar
359. Niwa, K, Perloff, JK, Bhuta, SM, Laks, H, Laks, H, Drinkwater, DC, Child, JS, et al. Structural abnormalities of great arterial walls in congenital heart disease: light and electron microscopic analyses. Circulation 2001; 103: 393400.Google Scholar
360. Bottio, T, Thiene, G, Tarzia, V, Rizzoli, G, Gerosa, G. Arterial switch operation, aortic root dilation, and long-term aortic valve competence. Ann Thorac Surg 2008; 86: 20252026.Google Scholar
361. Co-Vu, JG, Ginde, S, Bartz, PJ, Frommelt, PC, Tweddell, JS, Earing, MG. Long-term outcomes of the neoaorta after arterial switch operation for transposition of the great arteries. Ann Thorac Surg 2013; 95: 16541659.Google Scholar
362. Lalezari, S, Mahtab, EA, Bartelings, MM, Wisse, LJ, Hazekamp, MG, Gittenberger-de Groot, AC. The outflow tract in transposition of the great arteries: an anatomic and morphologic study. Ann Thorac Surg 2009; 88: 13001305.Google Scholar
363. Michalak, KW, Moll, JA, Moll, M, Dryzek, P, Moszura, T, Kopala, M, et al. The neoaortic root in children with transposition of the great arteries after an arterial switch operation. Eur J Cardiothorac Surg 2013; 43: 11011108.Google Scholar
364. Formigari, R, Toscano, A, Giardini, A, Gargiulo, G, Di Donato, R, Picchio, FM, et al. Prevalence and predictors of neoaortic regurgitation after arterial switch operation for transposition of the great arteries. J Thorac Cardiovasc Surg 2003; 126: 17531759.Google Scholar
365. Agnoletti, G, Ou, P, Celermajer, DS, Boudjemline, Y, Marini, D, Bonnet, D, et al. Acute angulation of the aortic arch predisposes a patient to ascending aortic dilatation and aortic regurgitation late after the arterial switch operation for transposition of the great arteries. J Thorac Cardiovasc Surg 2008; 135: 568572.Google Scholar
366. Jhang, WK, Shin, HJ, Park, JJ, Yun, TJ, Kim, YH, Ko, JK, et al. The importance of neo-aortic root geometry in the arterial switch operation with the trap-door technique in the subsequent development of aortic valve regurgitation. Eur J Cardiothorac Surg 2012; 42: 794799.Google Scholar
367. Murakami, T, Nakazawa, M, Momma, K, Imai, Y. Impaired distensibility of neoaorta after arterial switch procedure. Ann Thorac Surg 2000; 70: 19071910.Google Scholar
368. Fricke, TA, Brizard, CP, D’Udekem, Y, Konstantinov, IE. Aortic root and valve surgery after arterial switch operation. J Thorac Cardiovasc Surg 2012; 144: 12691271.Google Scholar
369. Marino, BS, Bridges, ND, Paridon, SM. Aortic insufficiency: indications for surgery in children. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 1998; 1: 147156.Google Scholar
370. Imamura, M, Drummond-Webb, JJ, McCarthy, JF, Mee, RB. Aortic valve repair after arterial switch operation. Ann Thorac Surg 2000; 69: 607608.Google Scholar
371. Alexi-Meskishvili, V, Photiadis, J, Nurnberg, JH. Replacement of the aortic valve after the arterial switch operation. Cardiol Young 2003; 13: 191193.Google Scholar
372. Hazekamp, MG, Schoof, PH, Suys, BE, Hutter, PA, Meijboom, EJ, Ottenkamp, J, et al. Switch back: using the pulmonary autograft to replace the aortic valve after arterial switch operation. J Thorac Cardiovasc Surg 1997; 114: 844846.Google Scholar
373. Vicente, W, Ferreira, CA, Klamt, JG, Manso, PH, Filho, OC, Carlotti, AP, et al. The switch back Ross operation: report of two cases with good medium-to-long-term follow-up. World J Pediatr Congenit Heart Surg 2012; 3: 244248.Google Scholar
374. Hourihan, M, Colan, SD, Wernovsky, G, Maheswari, U, Mayer, JE Jr, Sanders, SP. Growth of the aortic anastomosis, annulus, and root after the arterial switch procedure performed in infancy. Circulation 1993; 88: 615620.Google Scholar
375. Leobon, B, Belli, E, Ly, M, Kortas, C, Le Bret, E, Sigal-Cinqualbre, A, et al. Left ventricular outflow tract obstruction after arterial switch operation. Eur J Cardiothorac Surg 2008; 34: 10461050.Google Scholar
376. Kosaka, Y, Kurosawa, H, Nagatsu, M. Konno procedure using atrioventricular groove patch plasty after arterial switch operation. Ann Thorac Surg 2004; 78: 18541855.Google Scholar
377. Tamisier, D, Ouaknine, R, Pouard, P, Mauriat, P, Lefebvre, D, Sidi, D, et al. Neonatal arterial switch operation: coronary artery patterns and coronary events. Eur J Cardiothorac Surg 1997; 11: 810817.Google Scholar
378. Pedra, SR, Pedra, CA, Abizaid, AA, Braga, SL, Staico, R, Arrieta, R, et al. Intracoronary ultrasound assessment late after the arterial switch operation for transposition of the great arteries. J Am Coll Cardiol 2005; 45: 20612068.Google Scholar
379. Bartoloni, G, Bianca, S, Patane, L, Mignosa, C. Pathology of coronary narrowing after arterial switch operation: autopsy findings in two patients who died within 3 months of surgical treatment and review of the literature. Cardiovasc Pathol 2006; 15: 4954.Google Scholar
380. Bonnet, D, Bonhoeffer, P, Piechaud, JF, Aggoun, Y, Sidi, D, Planche, C, et al. Long-term fate of the coronary arteries after the arterial switch operation in newborns with transposition of the great arteries. Heart 1996; 76: 274279.Google Scholar
381. Tanel, RE, Wernovsky, G, Landzberg, MJ, Perry, SB, Burke, RP. Coronary artery abnormalities detected at cardiac catheterization following the arterial switch operation for transposition of the great arteries. Am J Cardiol 1995; 76: 153157.Google Scholar
382. Angeli, E, Formigari, R, Pace Napoleone, C, Oppido, G, Ragni, L, Picchio, FM, et al. Long-term coronary artery outcome after arterial switch operation for transposition of the great arteries. Eur J Cardiothorac Surg 2010; 38: 714720.Google Scholar
383. Raisky, O, Bergoend, E, Agnoletti, G, Ou, P, Bonnet, D, Sidi, D, et al. Late coronary artery lesions after neonatal arterial switch operation: results of surgical coronary revascularization. Eur J Cardiothorac Surg 2007; 31: 894898.Google Scholar
384. Bonhoeffer, P, Bonnet, D, Piechaud, JF, Stumper, O, Aggoun, Y, Villain, E, et al. Coronary artery obstruction after the arterial switch operation for transposition of the great arteries in newborns. J Am Coll Cardiol 1997; 29: 202206.Google Scholar
385. Hutter, PA, Bennink, GB, Ay, L, Raes, IB, Hitchcock, JF, Meijboom, EJ. Influence of coronary anatomy and reimplantation on the long-term outcome of the arterial switch. Eur J Cardiothorac Surg 2000; 18: 207213.Google Scholar
386. Angeli, E, Gerelli, S, Beyler, C, Lamerain, M, Rochas, B, Bonnet, D, et al. Bicuspid pulmonary valve in transposition of the great arteries: impact on outcome. Eur J Cardiothorac Surg 2012; 41: 248255.Google Scholar
387. Bonnet, D, Bonhoeffer, P, Sidi, D, Kachaner, J, Acar, P, Villain, E, et al. Surgical angioplasty of the main coronary arteries in children. J Thorac Cardiovasc Surg 1999; 117: 352357.Google Scholar
388. Raanani, E, Kogan, A, Shapira, Y, Sagie, A, Kornowsky, R, Vidne, BA. Surgical reconstruction of the left main coronary artery: fresh autologous pericardium or saphenous vein patch. Ann Thorac Surg 2004; 78: 16101613.Google Scholar
389. Bergoend, E, Raisky, O, Degandt, A, Tamisier, D, Sidi, D, Vouhé, P. Myocardial revascularization in infants and children by means of coronary artery proximal patch arterioplasty or bypass grafting: a single-institution experience. J Thorac Cardiovasc Surg 2008; 136: 298305.Google Scholar
390. Prêtre, R, Turina, MI. Surgical angioplasty of the left main coronary artery in non-atherosclerotic lesions. Heart 2000; 83: 9193.Google Scholar
391. Prifti, E, Bonacchi, M, Luisi, SV, Vanini, V. Coronary revascularization after arterial switch operation. Eur J Cardiothorac Surg 2002; 21: 111113.Google Scholar
392. Jonsson, A, Jensen, J, Olsson, A, Holm, P, Liska, J. Follow-up of patients operated on with arterial patch angioplasty of the left main coronary artery. Ann Thorac Surg 2006; 81: 12491255.Google Scholar
393. Dion, R, Verhelst, R, Matta, A, Rousseau, M, Goenen, M, Chalant, C. Surgical angioplasty of the left main coronary artery. J Thorac Cardiovasc Surg 1990; 99: 241249.Google Scholar
394. Mavroudis, C, Backer, CL, Duffy, CE, Pahl, E, Wax, DF. Pediatric coronary artery bypass for Kawasaki congenital, post arterial switch, and iatrogenic lesions. Ann Thorac Surg 1999; 68: 506512.Google Scholar
395. Mavroudis, C, Backer, CL, Muster, AJ, Pahl, E, Sanders, JH, Zales, VR, et al. Expanding indications for pediatric coronary artery bypass. J Thorac Cardiovasc Surg 1996; 111: 181189.Google Scholar
396. Kampmann, C, Kuroczynski, W, Trubel, H, Knuf, M, Schneider, M, Heinemann, MK. Late results after PTCA for coronary stenosis after the arterial switch procedure for transposition of the great arteries. Ann Thorac Surg 2005; 80: 16411646.Google Scholar
397. Abhaichand, R, Morice, MC, Bonnet, D, Sidi, D, Bonhoeffer, P. Stent supported angioplasty for coronary arterial stenosis following the arterial switch operation. Catheter Cardiovasc Interv 2002; 56: 278280.Google Scholar
398. El-Segaier, M, Lundin, A, Hochbergs, P, Jogi, P, Pesonen, E. Late coronary complications after arterial switch operation and their treatment. Catheter Cardiovasc Interv 2010; 76: 10271032.Google Scholar
399. Agnoletti, G, Bajolle, F, Bonnet, D, Sidi, D, Vouhé, P. Late coronary complications after arterial switch operation for transposition of great arteries. Clinical and therapeutic implications. Images Paediatr Cardiol 2005; 7: 111.Google Scholar
400. Sung, SC, Chang, YH, Lee, HD, Woo, JS. Left subclavian artery free graft as a salvage technique after failed coronary artery transfer in arterial switch operation. Eur J Cardiothorac Surg 2005; 27: 515516.Google Scholar
401. Legendre, A, Chantepie, A, Belli, E, Vouhé, PR, Neville, P, Dulac, Y, et al. Outcome of coronary artery bypass grafting performed in young children. J Thorac Cardiovasc Surg 2010; 139: 349353.Google Scholar
402. Robotin, MC, Bruniaux, J, Serraf, A, Uva, MS, Roussin, R, Lacour-Gayet, F, et al. Unusual forms of tracheobronchial compression in infants with congenital heart disease. J Thorac Cardiovasc Surg 1996; 112: 415423.Google Scholar
403. Toker, A, Tireli, E, Bostanci, K, Ozcan, V, Dayioglu, E. Uncommon complication of arterial switch operation: tracheobronchial compression. Ann Thorac Surg 2000; 69: 927929.Google Scholar
404. Worsey, J, Pham, SM, Newman, B, Park, SC, del Nido, PJ. Left main bronchus compression after arterial switch for transposition. Ann Thorac Surg 1994; 57: 13201322.Google Scholar
405. McElhinney, DB, Reddy, VM, Reddy, GP, Higgins, CB, Hanley, FL. Esophageal compression by the aorta after arterial switch. Ann Thorac Surg 1998; 65: 246248.Google Scholar
406. Gandhi, SK, Pigula, FA, Siewers, RD. Successful late reintervention after the arterial switch procedure. Ann Thorac Surg 2002; 73: 8893.Google Scholar
407. Corno, A, Giamberti, A, Giannico, S, Marino, B, Rossi, E, Marcelletti, C, et al. Airway obstructions associated with congenital heart disease in infancy. J Thorac Cardiovasc Surg 1990; 99: 10911098.Google Scholar
408. Kumar, A, Taylor, GP, Sandor, GG, Patterson, MW. Pulmonary vascular disease in neonates with transposition of the great arteries and intact ventricular septum. Br Heart J 1993; 69: 442445.Google Scholar
409. Damen, J, Hitchcock, JF. Reactive pulmonary hypertension after a switch operation. Successful treatment with glyceryl trinitrate. Br Heart J 1985; 53: 223225.Google Scholar
410. Kerstein, D, Levy, PS, Hsu, DT, Hordof, AJ, Gersony, WM, Barst, RJ. Blade balloon atrial septostomy in patients with severe primary pulmonary hypertension. Circulation 1995; 91: 20282035.Google Scholar
411. Blanc, J, Vouhé, P, Bonnet, D. Potts shunt in patients with pulmonary hypertension. N Engl J Med 2004; 350: 623.Google Scholar
412. Aziz, KU, Paul, MH, Rowe, RD. Bronchopulmonary circulation in d-transposition of the great arteries: possible role in genesis of accelerated pulmonary vascular disease. Am J Cardiol 1977; 39: 432438.Google Scholar
413. Wernovsky, G, Bridges, ND, Mandell, VS, Castaneda, AR, Perry, SB. Enlarged bronchial arteries after early repair of transposition of the great arteries. J Am Coll Cardiol 1993; 21: 465470.Google Scholar
414. Golej, J, Trittenwein, G, Marx, M, Schlemmer, M. Aortopulmonary collateral artery embolization during postoperative extracorporeal membrane oxygenation after arterial switch procedure. Artif Organs 1999; 23: 10381040.Google Scholar
415. Jowett, V, Derrick, G, Tsang, V, Marek, J. Coil occlusion of aortopulmonary collateral arteries before arterial switch procedure in an infant with transposition of the great arteries. Circ Cardiovasc Imaging 2008; 1: E17E18.Google Scholar
416. Irving, C, Chaudhari, M. Enlarged bronchial collateral artery complicating recovery after arterial switch for simple transposition of the great arteries. Interact CardioVasc Thorac Surg 2008; 7: 11761177.Google Scholar
417. Santoro, G, Carrozza, M, Russo, MG, Calabro, R. Symptomatic aorto-pulmonary collaterals early after arterial switch operation. Pediatr Cardiol 2008; 29: 838841.Google Scholar
Figure 0

Table 1 Classes of recommendations

Figure 1

Table 2 Levels of evidence

Figure 2

a

Figure 3

a

Figure 4

a

Figure 5

a

Figure 6

a

Figure 7

a

Figure 8

a

Figure 9

a

Figure 10

a

Figure 11

a

Figure 12

a

Figure 13

a

Figure 14

a

Figure 15

a

Figure 16

a

Figure 17

a

Figure 18