An Institutional Experience of Cardiac Transplantation for Congenital Heart Disease

therapies have not been trialled in children and there is little information about long-term survival in treated children. Methods: A multicentre, web-based Australian and New Zealand registry for children with PAH was established to better understand the profile of children with IPAH, aiming to describe demographics, presenting features, clinical course and outcomes of children aged three months to 18 years at time of PAH diagnosis, from 1/1/2002. A group of historic controls with IPAH diagnosed between 1970 and 1990 were used for comparison. Results: Of the 24 patients enrolled with IPAH, 54% are female and 75% Caucasian. The mean (SD) age at diagnosis was 9.3 (4.90) years and 50% were WHO functional class III at presentation. Baseline cardiac catheterisation data for both groups is shown. During follow-up, 20 (83%) subjects were treated with an endothelin receptor antagonist (exclusively Bosentan monohydrate), 17 (71%) with a phosphodiesterase 5 inhibitor (sildenafil) and 10 (42%) with either epoprostenol or iloprost; 19 (79%) are anticoagulated with warfarin. The mean duration of follow-up was 3.45 years, with an annualised mortality of 6%. Study endpoints of death/transplant occurred in 6/24, compared to 10/12 controls (p = .001). Severity of PAH from cardiac catheterisation was similar between patients and controls. Conclusions: Multiple medical therapies have resulted in improved survival for children with IPAH diagnosed during the current era. A better understanding of childhood IPAH will facilitate medical care, permit standardisation of therapeutic guidelines, and allow better representation in the healthcare arena. doi:10.1016/j.hlc.2011.05.572 569 An Institutional Experience of Cardiac Transplantation for Congenital Heart Disease K. Chee, M. Brown ∗ , D. Platts, S. McKenzie, A. Sharma, G. Javorsky The Prince Charles Hospital, Australia Background: Children with congenital heart diseases (CHD) are now surviving to adulthood but may deteriorate requiring cardiac transplantation. However, this treatment option has been associated with a high mortality. Method: A retrospective analysis of our heart/heart–lung transplant program was performed and patients with CHD were evaluated in relation to aetiology, surgical methods and outcome. Results: Since 1990, 2.2% (7/322) transplants were performed for CHD. Six out of seven heart alone and one in seven heart–lung transplantation. Mean age 29.5 years (range 9–45). Indication: tetralogy of fallot (three in seven), transposition great arteries (two in seven), tricuspid atresia (one in seven) and one double outlet hypoplastic right ventricle with pulmonary stenosis, VSD. Previous surgical episodes; mean of 2.4 (range 1–5). Mean bypass time was 4.2 hours and transfusion requirements were 15.4 units

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(range 3–39 units). Three patients required reopen for haemorrhage. Perioperative mechanical support was used in three patients (one BiVAD, one LVAD, and one IABP). Two out of seven (29%) patients died within 30 days of transplantation, related to bleeding complications. Both had four or more pre-transplant operations and their peritransplant transfusion requirements were 39 and 14 units. Eighty-three percent of patients had at least one episode of 3R rejection. Post transplant complications included stable stage II or III chronic kidney disease (2) and chronic myeloid leukaemia at 10 years (1). All survivors had a LVEF greater than 50% at last review. Mean follow up was 7.1 years. Conclusion: Transplantation in adult CHD poses significant challenges owing to their complex anatomy, previous operations and immune sensitisation. Cardiac transplantation in this patient population is a rare indication but has high perioperative/early mortality. doi:10.1016/j.hlc.2011.05.573 570 Arterial Ischaemic Stroke in Children with Heart Disease: Experience in a Single Centre Over 17 Years D. Hutchinson ∗ , M. Cheung, M. Cardamone, M. Mackay, C. Barnes, L. Coleman The Royal Children’s Hospital, Australia Background: Cardiac disease is the most common previously identified risk factor for Arterial Ischaemic Stroke (AIS) in children with limited data on the subgroups most at risk. Aims: To describe the spectrum of cardiac disorders, timing in relation to interventional procedures and outcome in children with both cardiac disease and AIS. Methods: One hundred and sixty three neonates and children aged <18 years with cardiac disease and stroke admitted to Royal Children’s Hospital Melbourne from 1993 to 2010 were retrospectively identified using ICD 9 and 10 searches. Results: Seventy-seven children (50 male) were identified with radiologically proven AIS. Fifty three percent were aged <1 year. Cardiac lesions included complex cyanotic congenital heart disease (n = 47, 61%), simple acyanotic lesions (n = 10, 13%), aortic lesions (n = 8, 10%), infective causes (n = 5, 6%), cardiomyopathies (n = 5, 6%) and primary arrhythmias (n = 3, 4%). Fifty-three patients (69%) had an arterial stroke within seven days of cardiac procedure. Strokes related to surgery type were reviewed with risk of AIS being highest with RV-PA conduit insertion (1.5%), BT shunt insertion (1.4%) and truncus arteriosis repair or Fontan completion (1.3%) and lowest with ASD (0.1%) and VSD closure (0.3%). Thirteen children (17%) died and 54 survivors (70%) have a neurological deficit. Conclusions: AIS is an important cause of morbidity and mortality in children with heart disease. The risk is greatest in children with complex cyanotic heart disease in the perioperative period. Further information is needed to identify

ABSTRACTS

Heart, Lung and Circulation 2011;20S:S156–S251