Background: Continuous intravenous Epoprostenol treatment has been shown to be beneficial in patients with severe pulmonary arterial hypertension (PAH). We report the experience of the UK Pulmonary Hypertension Service for Children treating 39 children with this therapy. Patients and methods: The patients were aged 4 months to 17 years (median 5.4 years), male: female ratio was 1: 1.2. Eleven children were treated with Epoprostenol alone. Twenty-eight were also treated with Sildenafil, Bosentan, Nifedipine or a combination of these. Twenty-five children had idiopathic PAH and 14 had PAH associated with congenital heart disease (9), connective tissue disease (2), chronic lung disease (2) or HIV (1). Cardiac catheter studies made before treatment began showed that in 26 patients the pulmonary artery pressure equalled or exceeded the systemic pressure. Thirty-four patients had a pulmonary vascular resistance (PVR) study with a mean PVR of 23 ± 12 units m2. After initial evaluation children were monitored by ECG, transthoracic echocardiography, weight, 6-minute-walk test and WHO functional class. Results: Follow-up was 1–90 months (mean 27). Twenty-four children remain stable and well, 8 have been transplanted and 7 died on treatment. There was no deterioration in the ECG; the 6-minute walk distance and right ventricular function assessed by echocardiography remained stable. WHO functional class improved from 3.6 to 2.6 during the first year (n = 38) (p < 0.001) and remained stable for up to 3 years (n = 17). Weight improved significantly (p < 0.05). A Kaplan Meier survival curve showed that survival was 94.4% at 1 year, 90.3% at 2 years and 83.9% at 3 years.

Conclusions: Epoprostenol treatment improved survival, clinical status and WHO functional class in children with severe PAH. Survival in the children on intravenous Epoprostenol with and without combination therapy was better than that described in adult studies (McLaughlin VV, et al. Circulation 2002; 106: 1477–1482).

The majority of arrhythmias presenting in infancy cardiovert readily or rapidly respond to conventional medical therapy. A small number prove highly refractory to anti-arrhythmic medications. Myocardial performance may be severely compromised by the combination of fast heart rate and negatively inotropic drugs. Some babies die. We have recently supported 2 babies with refractory arrhythmias on ECMO, both to pursue drug therapy and eventually, to support the circulation during radiofrequency ablation, with very successful results. The first patient was a 2.5 kg neonate presenting with collapse secondary to atrial ectopic tachycardia with a rate of 300/minute. Myocardial function was severely impaired. The arrhythmia was adenosine resistant and after iv amiodarone loading had no effect, esmolol infusion was started. This produced profound hypotension and the arrhythmia rapidly recommenced after DC cardioversion. In the face of such severe haemodynamic disturbance, VA ECMO was instituted. Further anti-arrhythmics were tried on-circulatory support, but the arrhythmia was incessant despite multiple DC cardioversions. Therefore radiofrequency ablation of the atrial ectopic focus was attempted on ECMO support. This was achieved uneventfully and the myocardial function rapidly improved, with decannulation 24 hours later. Unfortunately the arrhythmia recurred 2 weeks later, but was successfully treated by further ablation without ECMO. The child remains well with normal development on no medication. An 11-month-old baby presented to the GP with acute onset of lethargy and poor feeding and a heart rate of 350 bpm was noted! This was a broad complex tachycardia with independent p wave activity (confirmed with adenosine); ie ventricular tachycardia. Although initially well tolerated, the tachycardia resisted DC “cardioversion” even up to 60 J. Progressive and severe myocardial dysfunction and hypotension ensued, exacerbated by any attempts at drug therapy. The patient was therefore placed on VA ECMO. Some slowing of the ventricular rhythm was achieved with amiodarone and flecainide. The patient was then decannulated but the rapid arrhythmia recurred and ECMO was reinstituted. Electro-physiological mapping was then undertaken on ECMO support. NAVEX mapping identified a right ventricular outflow tract focus. This was resistant to conventional RF energy but was eventually successfully ablated with a “Cool-tip” catheter. Myocardial function improved rapidly, the patient was decannulated after 48 hours observation and there has been no recurrence of the arrhythmia since discharge. Although viewed as a very invasive technique, VA ECMO support here has prevented two otherwise unavoidable deaths in babies with conditions readily treated by radio-frequency techniques in older children. Not only did ECMO permit institution of aggressive drug therapy but also safely supported catheter interventions in very small patients. ECMO support should be considered early for small patients with refractory arrhythmias, before irreversible neurological compromise ensues. It could also be used electively to permit radiofrequency ablation in children whose size causes concern for safe catheter manipulation.

Background: Right ventricular (RV) impairment is a well recognized late sequelae of atrial inversion operation. Although conservative interventions as well as anatomic correction have been advocated late after Mustard or Senning procedures, heart transplantation remains the main treatment of patients with end-stage failure of the systemic RV. Aim of this study is to evaluate operative and postoperative results in patients undergoing heart transplantation for end-stage failure of the systemic RV following Mustard/Senning operations. Methods: Retrospective review of patients referred to the Regional Cardiothoracic Centre for cardiac transplantation was performed. Results: Since January 1987, 62 out of 567 patients underwent heart transplantation for congenital heart disease. Nine of these had a previous atrial inversion operation performed (6 Mustard, 3 Senning) for TGA. Mean age at time of atrial inversion operation was 16.9 ± 10.1 months. Time interval between atrial inversion and heart transplant was 22.0 ± 11.6 years. Mean age at heart transplant was 23.6 ± 10.8 years. Three patients were on inotropic support before the operation, while one was on ECMO support. Indication for heart transplantation was end-stage RV faiure (mean NYHA class 3.6 ± 0.5) with severe impaired function and severe tricuspid valve regurgitation in all patients. Results are shown in Table 1. At a mean follow up time of 21.5 ± 10.09 months, 71.5% of hospital survivors are alive. Conclusions: Heart transplantation for end-stage RV failure following atrial inversion operation is a high-risk procedure; however it remains the main treatment for this group of patients. The presence of a failing RV and previous surgery increases the operative mortality and exposes patients to late complications.

Purpose: Although intravascular ultrasound (IVUS) in children has been reported it is not widely used due to concerns over safety. Detailed analyses of measurements outlined by the ACC consensus statement on IVUS have not been reported in children. We report our safety and analysis data to date. Procedures: IVUS of the left anterior descending was performed with mechanised pullback concurrently with surveillance coronary angiography. Procedure and fluoroscopy screening times were compared with a second group of patients who experienced coronary angiography alone. Analysis was performed to measure vessel and lumen area, vessel and lumen diameters and intimal thickness for serial slices at fixed intervals from a distal identification point. Mean atheroma burden, mean maximum intimal thickness (MIT) and overall maximal intimal thickness were determined for each study. Results: Twenty-seven procedures were analysed. Table 1 shows patient demographics and information relating to IVUS analysis. Details of procedure and fluoroscopy time for both IVUS and coronary angiography groups are presented in Table 2. No complications were encountered. Routine coronary angiography was normal in all but one patient. Conclusions: We have demonstrated the safety of IVUS for coronary surveillance in children. When compared to coronary angiography alone procedure time and screening time are increased but we feel this is outweighed by the increased sensitivity of IVUS. Fifty-two percent of children have an IMT > 0.5 mm despite normal angiography. Such detailed analysis would appear central to future research into paediatric coronary disease.

Introduction: Significant variation exists in the perceived indications for balloon atrial septostomy in children with tricuspid atresia (TA). Many units advocate early, elective balloon atrial septostomy due to potential progressive narrowing of the interatrial opening, especially after the insertion of an arterial shunt. Our practice is to perform an atrial septostomy only in children with clinical and/or echocardiographic features of a restrictive interatrial communication. The aim of the study was to assess our practice by retrospective evaluation of patients born with tricuspid atresia with particular reference to the clinical fate of the atrial septum. More specifically the frequency of emergency atrial septostomy, the complication rate, the need for delayed septostomy or surgical resection and the longer term outcome were evaluated. Materials and methods: The study was a retrospective review (1980–2005) of the data on 49 live-born patients in our departmental database with a diagnosis of TA. One patient was excluded from the study due to referral to another centre and subsequent loss to follow up. By reviewing the medical notes of the remaining 48 patients we collected data on indications for, and timing of balloon atrial septostomy and subsequent complications and long term outcome. Results: The median time to follow up was 9.5 years (0.7–23.7). The diagnosis group was heterogeneous with 38 (79%) patients diagnosed with TA and normally related great arteries with or without pulmonary stenosis or atresia. The remaining 10 patients had more complex anatomy. Of the 48 patients, 5 (10%) were deemed to require balloon atrial septostomy for a clinically and/or echocardiographically restrictive atrial septum. Three procedures were performed within the first 24 hrs of life and 2 within the first month. One of the patients developed NEC following the septostomy and subsequently died of overwhelming sepsis. One patient required delayed surgical septectomy at 6 years of age, during a bidirectional Glenn operation. Of the remaining 42 patients there were 7 deaths (16%) but none were attributed to a restrictive atrial septum (1–at birth, severe acidosis and multi organ failure due to interrupted aortic arch and poor response to IV Prostin; 1–5 days with Group B streptococcal sepsis; 1–post operative sepsis following insertion of a BT shunt; 1–9 years, acute shunt obstruction; 1–withdrawal of care due to complex anatomy deemed to have a poor prognosis; 2-sudden death at home at 3½ years and 11 years). 26 patients required palliation with an arterial shunt (19–modified BT shunt, 6–Waterston Shunt, 1–ductal stent). None of the patients developed a clinically and/or echocardiographically restrictive atrial septum post procedure or during long term follow-up. Conclusions: We conclude that elective balloon atrial septostomy at presentation may not be necessary in patients born with tricuspid atresia. Accepting the limitations of retrospective data collection we also conclude that progressive narrowing of the interatrial septum at a later date is an unusual occurrence, even after an arterial shunt operation. We recommend that atrial septostomy should only be performed in patients who show obvious clinical and/or echocardiographic evidence of restrictive interatrial communication.

Background: Asymptomatic proximal coronary artery stenosis after arterial switch operation (ASO) is rare, but a potentially life-threatening condition, that is reported to appear in up to 7%. Angiography, although considered the state-of-the-art method of diagnosis, is an invasive method, but has limitations for diagnosing ostial stenosis. We report changes in Doppler flow profile and coronary flow reserve (CFR) in two asymptomatic patients (9- and 10-year old) with left main coronary artery (LMCA) ostial stenosis after ASO. Methods: Coronary flow was assessed by Transthoracic pulsed and colour-flow Doppler echocardiography (TTDE). CFR was measured in one patient using adenosine infusion (140 mcg/kg/min) over 4 minutes. CFR was calculated as the ratio of reactive hyperaemia to basal average peak velocity (APV). Both children were investigated with coronary angiography. They had myocardial Single-photon Emission Computed Tomography (SPECT), and magnetic resonance imaging (MRI) at rest and after reactive hyperaemia with adenosine infusion. Both patients had balloon dilatation and Cypher select (drug eluted) stent. Results: On echocardiogram a flame-like colour-flow diastolic signal was detected at the stenotic coronary ostia. The maximal spectral velocities during baseline conditions over the stenotic ostia were over 1.9 and 2.0 m/s (normal 30 ± 10 cm/s). The post-stenotic CFR was haemodynmically significant with value of 1.3, normal adult range 2.5–4. Coronary angiography showed a significant ostial stenosis 90% in both patients. Myocardial SPECT and MRI at rest/adenosine infusion were consistent with severe myocardial ischemia in the territory of the left coronary artery. Normal coronary angiography and coronary flow studies after stenting. Conclusion: We suggest that coronary artery flow assessment should be an integral part of the TTDE in the follow up of children with ASO. Serious coronary artery stenosis can be detected with TTDE. Assessment of CFR provides information of the physiological significance of the coronary stenosis.

Background: Ventricle-pulmonary artery connections are rare in patients after the Fontan procedure. However, these can cause significant long term problems. Hence the catheter occlusion of the anterograde flow in these patients would be beneficial. Further, in patients with a cavopulmonary shunt anterograde pulmonary blood flow is frequently maintained, but, in some patients, can cause significant volume loading of the heart or can complicate the subsequent Fontan procedure. Objective: To evaluate the use of interventional catheter closure of a ventricle-pulmonary artery communication in the setting of a cavopulmonary shunt or after the Fontan procedure. Patients and methods:This was a retrospective study at a tertiary referral centre. Eight patients (Age: 1.5–18 years, mean 7.8 years) underwent transcatheter closure of a ventricle-pulmonary artery communication. Indications were cardiac failure or persistent pleural effusions after cavopulmonary shunt (n = 2) or after Fontan (n = 3) and abolishing the volume load of the single ventricle prior to Fontan completion (n = 3). Results: Devices used were 17 mm Rashkind Umbrella device (n = 1), Amplatzer PDA device (n = 7) and Amplatzer ASD device (n = 1). One patient required two devices. There were no procedural complications. All 3 patients with prolonged pleural effusions (1 post CP shunt and 2 post Fontan) showed complete resolution of effusions between 4 and 10 days after catheter closure. Two patients underwent transcatheter occlusion for progressive ventricular dilatation and cardiac failure. The first patient was post Fontan and showed gradual improvement in ventricular function. The second patient after CP shunt died 48 hours post intervention (non procedure related cardiac death). Three patients underwent catheter closure to off-load the systemic ventricle prior to the Fontan procedure. The device had to be removed prior to release in 1 patient, due to unsatisfactory position. All 3 patients underwent successful completion of Fontan 6 weeks to 3 months post catheter. Conclusions: Transcatheter closure of ventricle-pulmonary artery communication is a safe and effective technique in the treatment of selected patients after CP shunt or Fontan procedure with early or late complications due to inappropriate pulmonary blood flow. This intervention should also be considered prior to the Fontan procedure in selected patients with ventricular overload.