Cardiac transplantation for infants with hypoplastic left heart syndrome

Orthotopic cardiac transplantation (OCT) has been our preferred treatment for infants with hypoplastic left heart syndrome (HLHS). This therapy was initially based on years of successful animal studies and later on continued as the results of transplantation proved encouraging. Infants with HLHS awaiting transplantation are maintained on prostaglandin E(1). Ductal stent placement may be necessary in some patients to maintain systemic perfusion. Others may require closed or open atrial septostomy. Shortage of donor organs accounts for 25% pretransplant mortality in our series. Over the last 10 years, 137 infants with HLHS underwent OCT. The operative mortality is 10%, and there have been 20 late deaths mostly due to rejection. The actuarial survival at 1 and 7 years following OCT is 84% and 72%, respectively. Three survivors required retransplantation at 1 month, 2 years and 7 years after initial OCT. Eight recipients developed moderate or severe graft coronary vasculopathy and only one patient was diagnosed with a neoplasm. In spite of few limitations, OCT is an effective therapy for HLHS with acceptable intermediate-term results