CARDIAC TRANSPLANTATION FOR INFANTS WITH HYPOPLASTIC LEFT-HEART SYNDROME

Orthotopic cardiac transplantation (OCT) has been our preferred treatm ent for infants with hypoplastic left heart syndrome (HLHS). This ther apy was initially based on years of successful animal studies and late r on continued as the results of transplantation proved encouraging. I nfants with HLHS awaiting transplantation are maintained on prostaglan din 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 mo rtality in our series. Over the last 10 years, 137 infants with HLHS u nderwent OCT. The operative mortality is 10%, and there have been 20 l ate deaths mostly due to rejection. The actuarial survival at 1 and 7 years following OCT is 84% and 72%, respectively. Three survivors requ ired retransplantation at 1 month, 2 years and 7 years after initial O CT. Eight recipients developed moderate or severe graft coronary vascu lopathy and only one patient was diagnosed with a neoplasm. In spite o f few limitations, OCT is an effective therapy for HLHS with acceptabl e intermediate-term results