We sought to assess left ventricular regional function in patients with and without left ventricular wall scar tissue in the long term after repair of an anomalous origin of the left coronary artery from the pulmonary artery.

A total of 20 patients aged 12.8±7.4 years were assessed 10 (0.5–17) years after the repair of an anomalous origin of the left coronary artery from the pulmonary artery; of them, 10 (50%) patients showed left ventricular wall scar tissue on current cardiac MRI. Left ventricular regional function was assessed by two-dimensional speckle-tracking echocardiography in 10 patients with scar tissue and 10 patients without scar tissue and in 10 age-matched controls.

In patients with scar tissue, MRI-derived left ventricular ejection fraction was significantly reduced compared with that in patients without scar tissue (51 versus 61%, p<0.05), and echocardiography-derived longitudinal strain was significantly reduced in five of six left ventricular areas compared with that in healthy controls (average relative reduction, 46%; p<0.05). In patients without scar tissue, longitudinal strain was significantly reduced in two of six left ventricular areas (average relative reduction, 23%; p<0.05) and circumferential strain was reduced in one of six left ventricular areas (relative reduction, 56%; p<0.05) compared with that in healthy controls.

Regional left ventricular function is reduced even in patients without left ventricular wall scar tissue late after successful repair of an anomalous origin of the left coronary artery from the pulmonary artery. This highlights the need for meticulous lifelong follow-up in all patients with a repaired anomalous origin of the left coronary artery from the pulmonary artery.

Right ventricular dysfunction in patients with tetralogy of Fallot and significant pulmonary regurgitation may lead to systolic dysfunction of the left ventricle due to altered ventricular interaction. We were interested in determining whether chronic pulmonary regurgitation affects the preload of the left ventricle. In addition, we wanted to study whether severe chronic pulmonary regurgitation would alter the preload of the left ventricle when compared with patients having preserved pulmonary valve annulus.

The study group comprised 38 patients with tetralogy of Fallot who underwent surgical repair between 1990 and 2003. Transannular patching was required in 21 patients to reconstruct the right ventricular outflow tract. Altogether, 48 age- and gender-matched healthy volunteers were recruited. Cardiac MRI was performed on all study patients to assess the atrial and ventricular volumes and function.

Severe pulmonary regurgitation (>30 ml/m2) was present in 13 patients, of whom 11 had a transannular patch, but only two had a preserved pulmonary valve annulus. The ventricular preload volumes from both atria were significantly reduced in patients with severe pulmonary regurgitation, and left ventricular stroke volumes (44.1±4.7 versus 58.9±10.7 ml/m2; p<0.0001) were smaller compared with that in patients with pulmonary regurgitation <30 ml/m2 or in controls.

In patients with tetralogy of Fallot, severe pulmonary regurgitation has a significant effect on volume flow through the left atrium. Reduction in left ventricular preload volume may be an additional factor contributing to left ventricular dysfunction.

Reports in the literature of treatment with recombinant tissue plasminogen activator following cardiac surgery are limited. We reviewed our experience to provide a case series of the therapeutic use of tissue plasminogen activator for the treatment of venous thrombosis in children after cardiac surgery. The data describe the morbidity, mortality, and clinical outcomes of tissue plasminogen activator administration for treatment of venous thrombosis in children following cardiac surgery.

The study was designed as a retrospective case series.

The study was carried out in a 25-bed cardiac intensive care unit in an academic, free-standing paediatric hospital.

All children who received tissue plasminogen activator for venous thrombosis within 60 days of cardiac surgery, a total of 13 patients, were included.

Data was collected, collated, and analysed as a part of the interventions of this study.

Patients treated with tissue plasminogen activator were principally young infants (median 0.2, IQR 0.07–0.58 years) who had recently (22, IQR 12.5–27.3 days) undergone cardiac surgery. Hospital mortality was high in this patient group (38%), but there was no mortality attributable to tissue plasminogen activator administration, occurring within <72 hours. There was one major haemorrhagic complication that may be attributable to tissue plasminogen activator. Complete or partial resolution of venous thrombosis was confirmed using imaging in 10 of 13 patients (77%), and tissue plasminogen activator administration was associated with resolution of chylous drainage, with no drainage through chest tubes, at 10 days after tissue plasminogen activator treatment in seven of nine patients who had upper-compartment venous thrombosis-associated chylothorax.

On the basis of our experience with administration of tissue plasminogen activator in children after cardiac surgery, tissue plasminogen activator is both safe and effective for resolution of venous thrombosis in this high-risk population.

The aims of this study were to investigate risk factors for the development of postoperative chylothorax following paediatric congenital heart surgery and to investigate the impact of a management guideline on management strategies and patient outcome.

All patients with chylothorax following cardiac surgery at the Royal Children’s Hospital, Melbourne, over a 48-month period beginning in January 2008 were identified. A control group, matched for age, date of surgery, and sex, was identified. To investigate potential risk factors, univariable and multivariable logistic regression models were constructed with paired analysis. To examine the effect of a standardised management protocol, data before and after the implementation of the guideline were compared.

In total, 121 cases of chylothorax were identified, with 121 controls, matched for age at surgery, date of surgery, and sex. The incidence of chylothorax was 5.23%. Increasing surgical complexity (univariable OR 0.17 for the least complex versus the most complex group, p=0.02), closed-heart surgeries (OR 0.07 for open versus closed, p<0.001), and redo chest incisions (OR 10.0 for redo versus virgin, p<0.001) were significantly associated with chylothorax. The standardised management protocol had no significant impact on either drainage duration or management strategy.

We have replicated the previously reported association between surgical complexity and chylothorax risk, and have shown, for the first time, that redo chest openings are also associated with a significantly increased risk. The implementation of a standardised management protocol in our institution did not result in a significant change in either chylothorax drainage duration or management strategy.

Although the right jugular vein approach for percutaneous pulmonary valve implantation is well described, there are no reports that describe a percutaneous pulmonary valve implantation through a left superior caval vein to coronary sinus pathway.

A 14-year-old female with tetralogy of Fallot, mesocardia, left superior caval vein draining into the coronary sinus, and hemiazygos continuation of the inferior caval vein underwent ventricular septal defect closure, with homograft insertion from the right ventricle to the pulmonary artery, patch augmentation of the left pulmonary artery, and creation of an atrial communication. Thereafter followed numerous catheterisations and interventions with stent implantation for stenosis of the left pulmonary artery and the homograft, as did device closure of the atrial communication. When she was a 12-year-old, the indications for a percutaneous pulmonary valve implantation were fulfilled and she underwent implantation of a 22 mm Melody® valve through the left superior caval vein. The extra-stiff exchange wire was pre-formed into a “U-spiral”-type configuration, according to the underlying anatomy, in order to provide a smooth route for the delivery of stents, to create the landing zone, and for the implantation of the Melody “ensemble”. The procedure was performed under deep sedation according to our standard protocol. The duration of the procedure was 172 min and the radiation time was 24.9 min.

On the basis of this unique experience, percutaneous pulmonary valve implantation is safe and feasible even in patients with unusual anatomy. Crucial is the “U-spiral” shaped configuration of the guide wire.

Paediatric hospital-associated venous thromboembolism is a leading quality and safety concern at children’s hospitals.

The aim of this study was to determine risk factors for hospital-associated venous thromboembolism in critically ill children following cardiothoracic surgery or therapeutic cardiac catheterisation.

We conducted a retrospective, case–control study of children admitted to the cardiovascular intensive care unit at Johns Hopkins All Children’s Hospital (St. Petersburg, Florida, United States of America) from 2006 to 2013. Hospital-associated venous thromboembolism cases were identified based on ICD-9 discharge codes and validated using radiological record review. We randomly selected two contemporaneous cardiovascular intensive care unit controls without hospital-associated venous thromboembolism for each hospital-associated venous thromboembolism case, and limited the study population to patients who had undergone cardiothoracic surgery or therapeutic cardiac catheterisation. Odds ratios and 95% confidence intervals for associations between putative risk factors and hospital-associated venous thromboembolism were determined using univariate and multivariate logistic regression.

Among 2718 admissions to the cardiovascular intensive care unit during the study period, 65 met the criteria for hospital-associated venous thromboembolism (occurrence rate, 2%). Restriction to cases and controls having undergone the procedures of interest yielded a final study population of 57 hospital-associated venous thromboembolism cases and 76 controls. In a multiple logistic regression model, major infection (odds ratio=5.77, 95% confidence interval=1.06–31.4), age ⩽1 year (odds ratio=6.75, 95% confidence interval=1.13–160), and central venous catheterisation (odds ratio=7.36, 95% confidence interval=1.13–47.8) were found to be statistically significant independent risk factors for hospital-associated venous thromboembolism in these children. Patients with all three factors had a markedly increased post-test probability of having hospital-associated venous thromboembolism.

Major infection, infancy, and central venous catheterisation are independent risk factors for hospital-associated venous thromboembolism in critically ill children following cardiothoracic surgery or cardiac catheter-based intervention, which, in combination, define a high-risk group for hospital-associated venous thromboembolism.

Acute kidney injury is a frequent complication after cardiac surgery with cardiopulmonary bypass in infants. Neutrophil gelatinase-associated lipocalin has been suggested to be a promising early biomarker of impending acute kidney injury. On the other hand, neutrophil gelatinase-associated lipocalin has been shown to be elevated in systemic inflammatory diseases without renal impairment. In this secondary analysis of data from our previous study on acute kidney injury after infant cardiac surgery, our hypothesis was that neutrophil gelatinase-associated lipocalin may be associated with surgery-related inflammation.

We prospectively enrolled 59 neonates and infants undergoing cardiopulmonary bypass surgery for CHD and measured neutrophil gelatinase-associated lipocalin in plasma and urine and interleukin-6 in the plasma. Values were correlated with postoperative acute kidney injury according to the paediatric Renal-Injury-Failure-Loss-Endstage classification.

Overall, 48% (28/59) of patients developed acute kidney injury. Of these, 50% (14/28) were classified as injury and 11% (3/28) received renal replacement therapy. Both plasma and urinary neutrophil gelatinase-associated lipocalin values were not correlated with acute kidney injury occurrence. Plasma neutrophil gelatinase-associated lipocalin showed a strong correlation with interleukin-6. Urinary neutrophil gelatinase-associated lipocalin values correlated with cardiopulmonary bypass time.

Our results suggest that plasma and urinary neutrophil gelatinase-associated lipocalin values are not reliable indicators of impending acute kidney injury in neonates and infants after cardiac surgery with cardiopulmonary bypass. Inflammation may have a major impact on plasma neutrophil gelatinase-associated lipocalin values in infant cardiac surgery. Urinary neutrophil gelatinase-associated lipocalin may add little prognostic value over cardiopulmonary bypass time.

The aim of this study was to describe atrial septal defect morphology in hypoplastic left heart syndrome, to report the incidence of restrictiveness and its relationship with defect morphology, to correlate restriction with midterm outcome, and to describe our interventional approach to restrictive defect.

From 2011 to 2015, 31 neonates with hypoplastic left heart syndrome underwent hybrid procedure with pulmonary artery banding and ductal stenting at our Institution. Restrictive physiology of the atrial septal defect was based on Doppler gradient >6 mmHg through the defect and on clinical signs of pulmonary hypertension. The mean gradient was then measured invasively. Restrictive defect occurred in 11/27 patients (40%). The restrictive group showed three ostium secundum defects (27%) and eight complex morphologies (73%). Conversely, in the non-restrictive group, we observed 11 ostium secundum defects (69%) and five complex morphologies (31%). Early balloon atrioseptostomy was required in three cases. Late restriction occurred in eight patients and was dealt with balloon dilation, stenting, or atrioseptectomy. There was no significant difference between restrictive and non-restrictive groups in terms of early or 12-month survival.

Complex morphologies were more frequently related to restrictiveness. Stenting technique has a crucial role, as the procedure carries a significant risk for stent migration. Effective treatment of restrictive atrial septal defect is related to a better outcome, as it leads to equalisation of survival between patients with and those without restrictive atrial septal defect.

The aim of this study was to evaluate the prevalence of acute kidney injury after first-stage surgical palliation in patients with a single ventricle and to explore associated risk factors and outcomes.

This single-centre retrospective study included neonates who underwent either Norwood or Hybrid procedure from 2008 to 2015 for a single ventricle. Postoperative acute kidney injury was defined using the paediatric risk, injury, failure, loss, end-stage renal disease (pRIFLE), criteria within 72 hours of the procedure.

Our cohort (n=48) underwent surgical palliation at a mean (SD) age of 12 (11) days. Postoperative acute kidney injury was diagnosed in 14 (29%) patients. The prevalence of acute kidney injury in the Hybrid group was 16% and 53% in the Norwood group. Infants who developed acute kidney injury underwent surgery at younger ages [6 (5–10) versus 10 (8–16) days, p=0.016], and had a higher peak lactate level in the initial 24 hours [5.9 (4.2–9.1) versus 3.4 (2.4–6.7), p=0.007]. Norwood procedure was significantly associated with acute kidney injury [odds ratio 11.7 (95% confidence interval 1.3–101.9), p=0.03]. ICU stay [38 (21–84) versus 16 (6–45) days, p=0.038] and time to extubation [204 (120–606) versus 72 (26–234) hours, p=0.014] were longer in those with acute kidney injury. The two patients who developed early postoperative renal failure as per pRIFLE died before discharge from associated comorbidities.

Acute kidney injury occurs in a third of the patients with single ventricle after surgical palliation but is mostly transient. Norwood, compared with Hybrid procedure, is a risk factor for postoperative acute kidney injury, which, in turn, is associated with longer ICU stay and time to extubation.

Patient-reported outcomes and epidemiological studies in adults with tetralogy of Fallot are lacking. Recruitment and longitudinal follow-up investigation across institutions is particularly challenging. Objectives of this study were to assess the feasibility of recruiting adult patients with tetralogy of Fallot for a patient-reported outcomes study, describe challenges for recruitment, and create an interactive, online tetralogy of Fallot registry.

Adult patients living with tetralogy of Fallot, aged 18–58 years, at the University of North Carolina were identified using diagnosis code query. A survey was designed to collect demographics, symptoms, history, and birth mother information. Recruitment was attempted by phone (Part I, n=20) or by email (Part II, n=20). Data analysis included thematic grouping of recruitment challenges and descriptive statistics. Feasibility threshold was 75% for recruitment and for data fields completed per patient.

In Part I, 60% (12/20) were successfully contacted and eight (40%) were enrolled. Demographics and birth mother information were obtained for all enrolled patients. In Part II, 70% (14/20) were successfully contacted; 30% (6/20) enrolled and completed all data fields linked to REDCap database; the median time for survey completion was 8 minutes. Half of the patients had cardiac operations/procedures performed at more than one hospital. Automatic electronic data entry from the online survey was uncomplicated.

Although recruitment (54%) fell below our feasibility threshold, enrolled individuals were willing to complete phone or online surveys. Incorrect contact information, privacy concerns, and patient-reported time constraints were challenges for recruitment. Creating an online survey and linked database is technically feasible and efficient for patient-reported outcomes research.

The aims of this study were to identify locations of births in Arizona with critical CHD, as well as to assess the current use of pulse-oximetry screening and capacities of birth centres to manage a positive screen.

Infants (n=487) with a potentially critical CHD were identified from the Arizona Department of Health Services from 2012 and 2013; charts were retrospectively reviewed. Diagnosis was confirmed using echocardiographies. ArcGIS was used to generate maps to visualise the location of treating facility and mother’s residence. Birth centres were surveyed to assess screening practices and capacities to manage critical CHD in 2015.

Of the 272 patients identified with critical CHD, 52% had been diagnosed prenatally. Patients travelled an average distance of 55.1 miles to their treating facility. Mortality was not related to prenatal diagnosis (p=0.30), living at a high elevation (p=0.82), or to distance travelled to the treating facility (p=0.68). Of 50 birth centres, 33 responded to the survey and all centres practiced critical CHD screening. A total of 25 centres could perform paediatric echocardiographies; 64% of these centres could digitally transmit echocardiograms. In all, 24 birth centres maintained access to prostaglandins.

Pulse-oximetry screening in newborns is currently implemented in the majority of Arizona hospitals. Although most centres could perform initial management steps following a positive screen, access to paediatric cardiology services was limited. Patients with critical CHD sometimes travelled a great distance to treating facilities. Digital transmission of echocardiograms or tele-echocardiography would reduce the distance travelled for the management of a positive screen, decrease the financial burden of transportation, and expedite care for critically ill neonates.

The adaptive changes of the foetal heart in intrauterine growth restriction can persist postnatally. Data regarding its consequences for early circulatory adaptation to extrauterine life are scarce. The aim of this study was to assess cardiac morphometry and function in newborns with late-onset intrauterine growth restriction to test the hypothesis that intrauterine growth restriction causes cardiac shape and functional changes at birth.

A comprehensive echocardiographic study was performed in 25 neonates with intrauterine growth restriction and 25 adequate-for-gestational-age neonates.

Compared with controls, neonates with intrauterine growth restriction had more globular ventricles, lower longitudinal tricuspid annular motion, and higher left stroke volume without differences in the heart rate. Neonates with intrauterine growth restriction also showed subclinical signs of diastolic dysfunction in the tissue Doppler imaging with lower values of early (e′) diastolic annular peak velocities in the septal annulus. Finally, the Tei index in the tricuspid annulus was higher in the intrauterine growth restriction group.

Neonates with history of intrauterine growth restriction showed cardiac remodelling and signs of systolic and diastolic dysfunction. Overall, there was a significant tendency to worse cardiac function results in the right heart. The adaptation to extrauterine life occurred with more globular hearts, higher stroke volumes but a similar heart rate compared to adequate-for-gestational-age neonates.

We aimed to study the dimensions, systolic and diastolic functions of the left ventricle; dimensions and elasticity of the aorta; and carotid intima-media thickness and flow-mediated dilatation of the brachial artery in mitral valve prolapse.

The study group consisted of 43 patients (mean age=13.3±3.9) and 42 healthy children (mean age=12.9±3.4). Left ventricular end-diastolic, end-systolic, left atrial diameters, interventricular septum, and left ventricular posterior wall thickness were measured. Ejection and shortening fractions were calculated by M-mode. Measurements were adjusted to the body surface area. Mitral annulus, and systolic and diastolic diameters of the aortic annulus and aorta at each level were obtained; z-scores, aortic strain, distensibility, stiffness index were calculated. Carotid intima-media thickness and flow-mediated dilatation were studied. Patients were classified as classical/non-classical mitral valve prolapse and younger/older patients.

Left ventricular end-systolic, end-diastolic, and left atrial diameters (p=0.009, p=0.024, p=0.001) and aortic z-scores at annulus, sinus valsalva, and sinotubuler junction were larger (p=0.008, p=0.003, p=0.002, respectively) in the mitral valve prolapse group. Aortic strain and distensibility increased and stiffness decreased at the ascending aorta in the patient group (p=0.012, 0.020, p=0.019, respectively). Classical mitral valve prolapse had lower strain and distensibility and higher stiffness of the aorta at sinus valsalva level (p=0.010, 0.027, 0.004, respectively). Carotid intima-media thickness was thinner in the patient group, especially in the non-classical mitral valve prolapse group (p=0.037). Flow-mediated dilatation did not differ among the groups.

Mitral valve prolapse is a systemic disease of the connective tissue causing enlarged cardiac chambers and increased elasticity of the aorta. Decreased carotid intima-media thickness in this group may indicate low atherosclerosis risk.

Early surgical management of common arterial trunk is well established and has good prognosis. Late diagnosis is less common. We reviewed late-diagnosed common arterial trunk management and prognosis for children in developing countries. We also discuss the need for prior catheterisation.

We reviewed all common arterial trunk patients managed by our humanitarian organization since 1996.

A total of 41 children with common arterial trunk were managed at a mean age of 3 years old. The lack of adequate facilities in developing countries explains the late management. The decision to proceed with surgery was based on clinical and radiological symptoms of persistent shunt, particularly a high cutaneous saturation level, regardless of catheterization – not carried out systematically. Eight children had to be withdrawn and 33 (80.5%) received operation – mean saturation 91%. The postoperative course was marked by pulmonary arterial hypertension requiring specific treatment in 30% of cases. The operative mortality was 1/33. The 32 children returned home without treatment after a mean post operative stay of 49 days and were followed up (mean FU 3.4 years, none lost to follow-up). At last contact, 1 child died six months after surgery, 1 child had a massive truncal valve insufficiency, 5 had a significant stenosis of the RV-PA tube, and 2 have had further surgery for tube replacement.

Late management and surgery of common arterial trunk is possible with good long-term results without prior hemodynamic examination up to an advanced childhood when signs of left-to-right shunt persist. A high saturation level (above 88%) seems to be a good operability criterion.