Orthotopic heart transplantation for congenital heart disease: An alternative for high-risk Fontan candidates?

Guido Michielon, Francesco Parisi, Cosimo Squitieri, Adriano Carotti, Giulia Gagliardi, Luciano Pasquini, Roberto M. Di Donato

Research output: Contribution to journalArticlepeer-review


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.

Original languageEnglish
Issue number10 SUPPL.
Publication statusPublished - Sep 9 2003


  • Congenital
  • Heart disease
  • Pulmonary circulation
  • Surgical aspects
  • Transplantation

ASJC Scopus subject areas

  • Physiology
  • Cardiology and Cardiovascular Medicine


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