title: Outcomes and risk factors for heart transplantation in children with congenital heart disease.
abstract: OBJECTIVE
  Heart transplantation in children with underlying congenital heart disease has traditionally been associated with inferior outcomes. We report our single institution experience.
  
  
  METHODS
  Between 1988 and 2013, 124 children with congenital heart disease underwent heart transplantation. Competing risks analysis modeled events after heart transplantation (retransplantation, death without retransplantation). Multivariable regression analysis examined risk factors affecting outcomes.
  
  
  RESULTS
  There were 76 male patients (61%); median age was 3.8 years (interquartile range, 0.6-11.5). Sixteen patients (13%) underwent primary heart transplantation, and 108 patients (87%) underwent prior surgical repair/palliation. Thirty-eight patients (31%) had 2 ventricles, and 86 patients (69%) had a single ventricle, including 33 (27%) who underwent a prior Fontan procedure. Competing risks analysis showed that at 10 years after heart transplantation, 13% of patients had undergone retransplantation, 43% of patients had died without retransplantation, and 44% of patients were alive without retransplantation. After retransplantation, 9 of 17 patients were alive and 3 of 17 patients had undergone a second retransplantation. Overall 15-year survival after initial heart transplantation was 41%. On multivariable analysis, risk factors for early-phase mortality were age less than 1 year (hazard ratio [HR], 7.2; 95% confidence interval [CI], 2.4-22.0; P < .001) and prolonged cardiopulmonary bypass (HR, 5.0; 95% CI, 2.1-11.8; P < .001). Risk factors for late-phase mortality were age more than 1 year (HR, 3.0; 95% CI, 1.1-7.7; P = .025) and donor-recipient race mismatch (HR, 2.2; 95% CI, 1.2-4.1; P = .016). Survival was not affected by era, underlying anomaly, prior Fontan procedure, sensitization, or pulmonary artery augmentation.
  
  
  CONCLUSIONS
  The outcomes of heart transplantation in children with congenital heart disease have not improved in the current era. Survival was not affected by the underlying anomaly, prior Fontan procedure, or sensitization. Strategies to improve the outcomes in patients with congenital heart disease may need to address selection criteria, transplantation timing, and pretransplant and post-transplant care. The effect of donor-recipient race mismatch warrants further investigation and might affect organ allocation algorithms or immunosuppression management.
authors.affiliation: Division of Cardiothoracic Surgery, Children's Healthcare of Atlanta, Emory University School of Medicine, Atlanta, Ga. Electronic address: balsoufi@hotmail.com.
authors.forename: Bahaaldin
authors.initials: B
authors.lastname: Alsoufi
authors.affiliation: Division of Cardiology, Department of Pediatrics, Children's Healthcare of Atlanta, Emory University School of Medicine, Atlanta, Ga.
authors.forename: Shriprasad
authors.initials: S
authors.lastname: Deshpande
authors.affiliation: Division of Cardiology, Department of Pediatrics, Children's Healthcare of Atlanta, Emory University School of Medicine, Atlanta, Ga.
authors.forename: Courtney
authors.initials: C
authors.lastname: McCracken
authors.affiliation: Division of Cardiothoracic Surgery, Children's Healthcare of Atlanta, Emory University School of Medicine, Atlanta, Ga.
authors.forename: Brian
authors.initials: B
authors.lastname: Kogon
authors.affiliation: Division of Cardiology, Department of Pediatrics, Children's Healthcare of Atlanta, Emory University School of Medicine, Atlanta, Ga.
authors.forename: Robert
authors.initials: R
authors.lastname: Vincent
authors.affiliation: Division of Cardiology, Department of Pediatrics, Children's Healthcare of Atlanta, Emory University School of Medicine, Atlanta, Ga.
authors.forename: William
authors.initials: W
authors.lastname: Mahle
authors.affiliation: Division of Cardiothoracic Surgery, Children's Healthcare of Atlanta, Emory University School of Medicine, Atlanta, Ga.
authors.forename: Kirk
authors.initials: K
authors.lastname: Kanter
mesh_terms: D002675:Child, Preschool; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications*; D016027:Heart Transplantation / Q000009:adverse effects / Q000401:mortality; D006801:Humans; D008297:Male; D015999:Multivariate Analysis; D012307:Risk Factors; D016896:Treatment Outcome
keywords: congenital heart disease; heart transplantation