Transplantation in complex congenital heart disease

Three hundred and forty-five pediatric patients underwent orthotopic heart transplantation at Loma Linda University Medical Center throughout August 1 999. Seventy-five percent of these patients had the diagnosis of congenital heart disease as the primary indication for transplantation. Two hundred a nd fifty-seven were infants and of these, 91 were neonates. Forty-nine perc ent had the primary diagnosis of hypoplastic left heart syndrome or its equ ivalent. Thirty patients had variant situs and 15 of these had situs invers us. Seventy-seven children had undergone one or more previous cardiac and/o r thoracic procedures. There appeared to be a survival advantage with neona tal as compared to infant heart transplants (77% vs. 63%);. Technical consi derations for reconstruction have made congenital heart transplantation a l ow perioperative risk factor as compared to the child with cardiomyopathy. Attention to (1) recipient selection; (2) adequate en bloc removal of the h eart and accompanying vessels; (3) use of low-flow bypass techniques with l imited circulatory rest; and (4) multidisciplinary management have been the fundamental approach at this institution. Although challenging, high perio perative survival and long-term success can be achieved with heart transpla ntation in patients with high-risk or inoperable congenital heart disease. (C) 2000 Elsevier Science Ireland Ltd. All rights reserved.