Results of orthotopic heart transplantation for failed palliation of hypoplastic left heart

Previous studies have indicated that results for orthotopic heart transplantation (OHT) in patients with surgically palliated hypoplastic left heart (HLHS) are worse compared with patients with other forms of congenital heart disease (CHD) or acquired cardiomyopathy (CM) as well as those undergoing primary OHT for HLHS. In light of the decreasing donor pool for transplantation and increasing numbers of palliated HLHS patients with improving survival, we sought to review our results for OHT in surgically palliated HLHS patients and failing Glenn or Fontan circulations. We conducted a single centre, retrospective study of patients undergoing OHT from 2000 to 2011. Patients who were transplanted following any of the three stages of palliation were included. Indications for OHT were severe impairment of systemic right ventricular (RV) function with/without significant atrioventricular (AV) valve regurgitation or failure of Fontan physiology. The primary outcome of interest was survival; the secondary outcomes examined were the incidence of post-transplant RV failure and the need for extracorporeal membrane oxygenation (ECMO) support. A total of 209 patients were transplanted during the study period. Of these, 16 were surgically palliated HLHS patients, 1 following Norwood I, 4 post-Fontan and 11 post-Glenn. Thirty-one patients had non-HLHS CHD and 154 patients had forms of acquired CM. Preoperative patient characteristics including age, weight and donor/recipient weight ratio were similar across groups, though the incidence of pulmonary hypertension (PHT) was higher in the CM group. Thirty-day survival was 100% in the palliated HLHS patients (vs 98.1% for the CM group), with 1- and 5-year Kaplan-Meier survivals of 100 and 87.5% (P = 0.393 vs CM; log-rank test). Intensive care unit stay was comparable with transplanted CM patients as was the incidence of RV failure and ECMO post-OHT. Our results suggest that good early and mid-term outcomes following OHT in surgically palliated HLHS are achievable. These findings have implications for the optimal strategy and timing for managing palliated patients with HLHS as well as for counselling parents and affected children.