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The effect of age, diagnosis, and previous surgery in children and adults undergoing heart transplantation for congenital heart disease
JACC - Journal of the American College of Cardiology, 07/02/09

Lamour JM et al. – Pts undergoing transplantation for congenital heart disease (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.

• Study of outcomes and risk factors for mortality after HT for CHD in infants, children, and adults
• Analysis of 488 pts transplanted for CHD from combined Pediatric Heart Transplant Study (1993-2002, n = 367) and the Cardiac Transplant Registry Database (1990-2002, n = 121)
• Primary diagnoses: single ventricle (36%), d-transposition of great arteries (12%), right ventricular outflow tract lesions (10%), l-transposition of great arteries (8%), ventricular/atrial septal defects (8%), left ventricular outflow obstruction (8%), and other (18%)
• Before HT, ≥1 operation for 93% of pts

• Median age at HT: 12.4 yrs
• Survival at 3 mo post-HT significantly worse in CHD pts versus children with cardiomyopathy, but not adults with cardiomyopathy (86%, 94%, and 91%, respectively)
• No difference in conditional 3-mo survival among the 3 groups
• 5-yr survival: 80%
• Risk factors for early mortality: older recipient age, older donors with longer ischemic times, and pre-HT Fontan operations
• Predicted survival in Fontan pts lower (77% and 70% at 1 and 5 yrs) vs non-Fontan pts (88% and 81% at 1 and 5 yrs)
• Risk factors for constant phase mortality: younger recipient age, higher transpulmonary gradient, cytomegalovirus mismatch at HT, and earlier classic Glenn operation

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