The effect of age, diagnosis, and previous surgery in children and adults undergoing heart transplantation for congenital heart disease

J Am Coll Cardiol. 2009 Jul 7;54(2):160-5. doi: 10.1016/j.jacc.2009.04.020.


Objectives: We 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.

Background: CHD is considered a risk factor for mortality after HT, yet this unique group of patients represents a spectrum of complexity.

Methods: There 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.

Results: The 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.

Conclusions: Patients 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.

Publication types

  • Comparative Study
  • Multicenter Study

MeSH terms

  • Adolescent
  • Adult
  • Age Distribution
  • Age Factors
  • Cardiac Surgical Procedures / methods
  • Child
  • Child, Preschool
  • Coronary Angiography / methods*
  • Echocardiography / methods*
  • Follow-Up Studies
  • Heart Defects, Congenital / diagnosis
  • Heart Defects, Congenital / mortality*
  • Heart Defects, Congenital / surgery
  • Heart Transplantation / methods*
  • Humans
  • Infant
  • Kaplan-Meier Estimate
  • Middle Aged
  • Retrospective Studies
  • Risk Factors
  • Survival Rate / trends
  • Time Factors
  • Treatment Outcome
  • United States / epidemiology
  • Young Adult