title: Cardiac transplantation after the Fontan or Glenn procedure.
abstract: OBJECTIVE
  The purpose of this study was to review the clinical course and outcome of cardiac transplantation after a failed Glenn or Fontan procedure.
  
  
  BACKGROUND
  Late complications of the Glenn or Fontan procedure, including ventricular failure, cyanosis, protein-losing enteropathy, thromboembolism, and dysrhythmias often lead to significant morbidity and mortality. If other therapies are ineffective, cardiac transplantation is the only therapeutic recourse. Transplantation in this unique population presents significant challenges in the operative and perioperative periods.
  
  
  METHODS
  The anatomic diagnoses, previous operations, clinical status, and indications for transplantation were characterized in patients transplanted after a Glenn or Fontan procedure. Outcomes after transplantation, including postoperative complications and mortality, were reviewed. Comparisons were made between survivors and nonsurvivors.
  
  
  RESULTS
  Primary orthotopic cardiac transplantation was performed in 35 patients (mean age 15.7 +/- 8.5 years) with a mean follow-up of 54 +/- 46 months. A total of 11 patients had undergone a Glenn shunt and 24 patients a Fontan procedure. Indications for transplantation were a combination of causes including ventricular dysfunction, failed Fontan physiology, and/or cyanosis. Ten patients died <or=2 months after transplantation; nine of the deaths occurred in the Fontan patients. Overall, one-year survival was 71.5%, and five-year survival was 67.5%. Survival was not significantly different between patients transplanted after a Glenn or Fontan procedure and patients transplanted for other etiologies.
  
  
  CONCLUSIONS
  Cardiac transplantation can be performed successfully in patients with end-stage congenital heart disease after a Glenn or Fontan procedure, with outcomes similar to transplantation for end-stage heart failure secondary to other etiologies.
authors.affiliation: Department of Pediatrics, College of Physicians & Surgeons, Columbia University, New York, New York, USA.
authors.forename: K Anitha
authors.initials: KA
authors.lastname: Jayakumar
authors.forename: Linda J
authors.initials: LJ
authors.lastname: Addonizio
authors.forename: Maryanne R
authors.initials: MR
authors.lastname: Kichuk-Chrisant
authors.forename: Mark E
authors.initials: ME
authors.lastname: Galantowicz
authors.forename: Jacqueline M
authors.initials: JM
authors.lastname: Lamour
authors.forename: Jan M
authors.initials: JM
authors.lastname: Quaegebeur
authors.forename: Daphne T
authors.initials: DT
authors.lastname: Hsu
mesh_terms: D000293:Adolescent; D000328:Adult; D006348:Cardiac Surgical Procedures / Q000379:methods*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000401:mortality* / Q000601:surgery*; D016027:Heart Transplantation / Q000706:statistics & numerical data*; D006801:Humans; D007223:Infant; D008297:Male; D011183:Postoperative Complications; D016019:Survival Analysis; D017211:Treatment Failure; D014481:United States / Q000453:epidemiology