Ethics of Cardiac Transplantation in Hypoplastic Left Heart Syndrome

Abstract

There has long been debate among experts in the care of infants born with hypoplastic left heart syndrome (HLHS) as to whether orthotopic heart transplantation or cardiac reconstruction is preferable as the primary approach [1, 15]. Many centers now favor the reconstructive techniques (Norwood-type procedure); however, several centers continue to offer cardiac transplantation as a primary treatment. By the late 1990s, approximately 50% of children born with HLHS in the United States underwent cardiac reconstruction (studies indicate that approximately 35% of infants born with HLHS in the United States underwent cardiac reconstruction and survived to hospital discharge [9, 14] and that the rate of survival to discharge postreconstruction was approximately 70% [5, 6, 12, 17, 20, 27, 28], yielding a calculated value of 50% undergoing reconstruction), approximately 5% underwent cardiac transplantation (with an additional 2% dying while on the transplant waiting list) [9, 10, 14], and the remaining patients (approximately 45%) received palliative treatment (previously referred to as comfort care without surgery [18]). More recent data have not been published; therefore, the current percentages treated with each option is unknown.