title: Heart transplantation for adults with congenital heart disease: analysis of the United network for organ sharing database.
abstract: BACKGROUND
  Congenital heart disease (CHD) in the adult is an uncommon indication for heart transplantation but has been increasing. We assessed survival and predictors of death after heart transplantation for adults with CHD.
  
  
  METHODS
  Adult primary heart transplant recipients (aged > 17 years) reported to the United Network for Organ Sharing (1987 to 2006) were reviewed and categorized by diagnosis of CHD vs other diagnoses. Kaplan-Meier survival analysis and Cox regression modeling were performed.
  
  
  RESULTS
  During the study period, 35,334 adults underwent primary heart transplantation, and 689 (2%) had CHD. Adult CHD recipients had longer mean waiting list time (218 vs 195 days; p = 0.004), longer ischemic time (3.5 vs 2.9 hours, p < 0.0001), and were more likely to have pretransplant pulmonary vascular resistance exceeding 4 Woods Units (62% vs 51%, p < 0.0001) vs other recipients. Thirty-day mortality was 16% vs 6% (p < 0.0001), although Kaplan-Meier survival did not differ between groups (p = 0.92) out to 10 years. Ischemic time (hazard ratio [HR], 1.2; 95% confidence interval [CI], 1.02 to 1.35; p = 0.02), African American race (HR, 1.9; 95% CI, 1.04 to 3.58; p = 0.03), and pulmonary vascular resistance exceeding 4 Woods Units (HR, 1.5; 95% CI, 1.01 to 2.19; p = 0.04) were predictors of death for adult CHD recipients.
  
  
  CONCLUSIONS
  Heart transplantation for adults with CHD is effective and has good long-term prognosis. The 30-day mortality rate is high, but 5- and 10-year survival is not statistically different from patients without CHD.
authors.affiliation: Division of Cardiac Surgery, The Johns Hopkins Medical Institutions, Baltimore, Maryland, USA.
authors.forename: Nishant D
authors.initials: ND
authors.lastname: Patel
authors.forename: Eric S
authors.initials: ES
authors.lastname: Weiss
authors.forename: Jeremiah G
authors.initials: JG
authors.lastname: Allen
authors.forename: Stuart D
authors.initials: SD
authors.lastname: Russell
authors.forename: Ashish S
authors.initials: AS
authors.lastname: Shah
authors.forename: Luca A
authors.initials: LA
authors.lastname: Vricella
authors.forename: John V
authors.initials: JV
authors.lastname: Conte
mesh_terms: D000293:Adolescent; D000328:Adult; D002423:Cause of Death; D016208:Databases, Factual; D005260:Female; D006330:Heart Defects, Congenital / Q000401:mortality / Q000601:surgery*; D006333:Heart Failure / Q000150:complications / Q000401:mortality; D016027:Heart Transplantation / Q000401:mortality* / Q000639:trends; D017052:Hospital Mortality; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications / Q000401:mortality; D053208:Kaplan-Meier Estimate; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D011183:Postoperative Complications / Q000401:mortality*; D012042:Registries; D012189:Retrospective Studies; D012307:Risk Factors; D014481:United States; D014655:Vascular Resistance; D014850:Waiting Lists; D052096:Warm Ischemia / Q000401:mortality; D055815:Young Adult