title: Orthotopic heart transplantation for failing single ventricle physiology.
abstract: OBJECTIVE
  Evaluation of incremental risk factors for early mortality in children undergoing orthotopic heart transplantation (OHT) for failing single ventricle physiology.
  
  
  METHODS
  Between 1988 and 2002, 25 patients (mean age 9.3+/-7.1 years) underwent OHT for complex congenital heart disease (CHD) with a functional right (15 patients) or left (10 patients) single ventricle. Palliative staging towards Fontan completion had been previously accomplished in 22 patients (88%). Transition to OHT occurred from a shunt stage in 10, a bi-directional cavopulmonary anastomosis (BDG) stage in nine, and after Fontan failure in six patients.
  
  
  RESULTS
  Thirty-day survival was 68.0+/-9.3% with no additional mortality up to 14.1 years. OHT following BDG staging exhibited 100% long-term survival, as opposed to 66.7+/-15.7% for OHT after systemic-to-pulmonary shunt, and 33.3+/-19.2% for OHT following failing Fontan (p=0.032). Regression logistic modelling indicated failing Fontan circulation as predictor of higher mortality after OHT (p=0.041). Reintervention was necessary in four patients 40+/-11 months after OHT to address residual superior vena cava (two) and isthmic (two) stenosis. Overall freedom from reintervention was 88.3+/-8.1% at 5 years.
  
  
  CONCLUSIONS
  OHT for structural CHD with single ventricle physiology entails substantial early mortality while BDG enables the best transition to heart transplant. OHT should be considered in the decision-making process as an alternative to Fontan completion in high-risk candidates, since rescue-OHT after failing Fontan seems unwarranted.
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D018729:Fontan Procedure; D006330:Heart Defects, Congenital / Q000503:physiopathology / Q000601:surgery*; D016027:Heart Transplantation / Q000379:methods*; D006352:Heart Ventricles / Q000503:physiopathology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D016015:Logistic Models; D008297:Male; D010166:Palliative Care; D011183:Postoperative Complications; D012086:Reoperation; D012189:Retrospective Studies; D012307:Risk Factors; D016019:Survival Analysis; D016896:Treatment Outcome