Results of heart transplantation following failed staged palliation of hypoplastic left heart syndrome and related single ventricle anomalies

OBJECTIVES: Multistage palliation is the mainstay management strategy of children with hypoplastic left heart syndrome (HLHS) and related single ventricle anomalies. If this palliation strategy fails, heart transplantation (HT) is required. The results of HT in children who had a prior Norwood operation are reportedly poor due to several immune, clinical and anatomical risk factors. We report our institutional outcomes following HT in children who had a prior Norwood operation. METHODS: Between 1994 and 2013, 107 children with congenital heart disease underwent HT. We examined early and late outcomes in our study cohort of children who had a prior Norwood operation (n = 24), and analysed risk factors affecting survival. Survival was subsequently compared with a control group of 83 children with congenital heart disease without a prior Norwood operation who received HT. RESULTS: Twenty-four children with a prior Norwood operation underwent HT. The majority (22/24, 92%) had HLHS. Children were listed following Norwood (n = 2, 8.3%), Glenn (n = 17, 70.8%) or Fontan (n = 5, 20.8%) operation. Ten (42%) patients had panel reactive antibodies (PRAs) > 10%. Median age at listing was 2.7 (range 0.4-16.8) years and median age at the time of HT was 3.0 (range 0.6-16.8) years, with the median waiting list duration of 63.5 (range 1-554) days. Hospital mortality was 1/24 (4%). Overall parametric survival estimates at 1, 5 and 10 years were 85, 65 and 52%. Survival was not affected by listing status, last palliation stage, age or high PRA. The only significant factor affecting survival was the later era in our series with significantly superior 5-year survival (100 vs 42%, P = 0.0003). Overall survival was comparable with the control group of children with congenital heart disease and no prior Norwood operation (52 vs 53% at 10 years, P = 0.97). Overall, 3 of 24 patients required retransplantation with only one late survivor. CONCLUSIONS: Children failing multistage palliation of HLHS may require HT, often following the Glenn operation. HT results in this group are comparable with those in other children with congenital heart disease. Improvements in pretransplant management, immune suppression and outpatient care in the later era might have specifically benefited this particularly risky group of patients.