• Jacqueline M Lamour, Kirk R Kanter, David C Naftel, Maryanne R Chrisant, William R Morrow, Barry S Clemson, James K Kirklin, Cardiac Transplant Registry Database, and Pediatric Heart Transplant Study.
• Department of Pediatric Cardiology, Columbia University, New York, NY, USA. jlamour@montefiore.org
• J. Am. Coll. Cardiol. 2009 Jul 7; 54 (2): 160-5.

ObjectivesWe sought to evaluate the outcomes and identify risk factors for mortality after heart transplantation (HT) for congenital heart disease (CHD) in infants, children, and adults.BackgroundCHD is considered a risk factor for mortality after HT, yet this unique group of patients represents a spectrum of complexity.MethodsThere were 488 patients transplanted for CHD from the combined Pediatric Heart Transplant Study (1993 to 2002, n = 367) and the Cardiac Transplant Registry Database (1990 to 2002, n = 121) who were analyzed.ResultsThe median age at HT was 12.4 years. Primary diagnosis included single ventricle (36%), d-transposition of the great arteries (12%), right ventricular outflow tract lesions (10%), l-transposition of the great arteries (8%), ventricular/atrial septal defects (8%), left ventricular outflow obstruction (8%), and other (18%). Ninety-three percent of patients had at least 1 operation before HT. Survival at 3 months post-HT was significantly worse in CHD patients versus children with cardiomyopathy, but not adults with cardiomyopathy (86%, 94%, and 91%, respectively). There was no difference in conditional 3-month survival among the 3 groups. Five-year survival was 80%. Risk factors for early mortality were older recipient age, older donors with longer ischemic times, and pre-HT Fontan operations. Predicted survival in Fontan patients was lower (77% and 70% at 1 and 5 years) versus non-Fontan patients (88% and 81% at 1 and 5 years). Risk factors for constant phase mortality included younger recipient age, higher transpulmonary gradient, cytomegalovirus mismatch at HT, and earlier classical Glenn operation.ConclusionsPatients undergoing transplantation for CHD have a good late survival if they survive the early post-operative period. Risk factors for reduced survival are older age at transplant and a previous Fontan operation.

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