title: Orthotopic heart transplantation for congenital heart disease: an alternative for high-risk fontan candidates?
abstract: OBJECTIVE
  Evaluation of incremental risk factors for early mortality in children undergoing orthotopic heart transplantation (OHT) for congenital heart disease.
  
  
  METHODS
  Between 1988 and 2002, 43 patients (mean age 9.1+/-7.2 years) underwent 44 OHT for complex TGA (6), DORV (4), single ventricle (21), and other end-stage structural heart disease (11). Two discernible ventricular chambers were present in 18 pts (41.8%). Previous reconstructive or palliative procedures had been previously accomplished in 35 pts (83.3%), including atrial switch (5), systemic-to-pulmonary shunts (10), cavopulmonary anastomosis (9), Fontan completion (6), and others (5).
  
  
  RESULTS
  30-day survival for the 2-ventricle subgroup was 94.4+/-5.4% compared with 67.2+/-9.5% for the single ventricle subgroup (P=0.04) (overall 78.6%+/-3.3%). OHT following single ventricle staging to bi-directional cavopulmonary anastomosis exhibited 100% early survival, as opposed to 62.5+/-17.1% for OHT after systemic-to-pulmonary shunts, and 33.3+/-19.2% for OHT following failing Fontan (P=0.010). HLHS diagnosis (0.0085) and failing Fontan (P=0.003) were identified as independent predictors of early mortality by regression logistic modeling, while Fontan stage represented the only predictor of overall mortality by Cox proportional hazard. Overall 10-year survival was 54.3+/-11%.
  
  
  CONCLUSIONS
  OHT for structural congenital heart disease with single ventricle physiology entails substantial early mortality and bi-directional cavopulmonary anastomosis 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.
authors.affiliation: Dipartimento Medico-Chirurgico di Cardiochirurgia e Cardiologia Pediatrica, DMCCP, Ospedale Pediatrico Bambino Gesù, Roma, Italy. guido.michielon@tin.it
authors.forename: Guido
authors.initials: G
authors.lastname: Michielon
authors.forename: Francesco
authors.initials: F
authors.lastname: Parisi
authors.forename: Cosimo
authors.initials: C
authors.lastname: Squitieri
authors.forename: Adriano
authors.initials: A
authors.lastname: Carotti
authors.forename: Giulia
authors.initials: G
authors.lastname: Gagliardi
authors.forename: Luciano
authors.initials: L
authors.lastname: Pasquini
authors.forename: Roberto M
authors.initials: RM
authors.lastname: Di Donato
mesh_terms: D000293:Adolescent; D002648:Child; D005260:Female; D018729:Fontan Procedure; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000601:surgery*; D016027:Heart Transplantation / Q000009:adverse effects / Q000401:mortality*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D010166:Palliative Care; D012086:Reoperation; D012307:Risk Factors; D016019:Survival Analysis; D017211:Treatment Failure