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(Circulation. 2003;108:II-140.)
© 2003 American Heart Association, Inc.

Surgery for Congenital Heart Disease

Orthotopic Heart Transplantation for Congenital Heart Disease: An Alternative for High-Risk Fontan Candidates?

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

From the Dipartimento Medico-Chirurgico di Cardiochirurgia e Cardiologia Pediatrica, DMCCP, Ospedale Pediatrico Bambino Gesù, Roma, Italy.

Correspondence to Guido Michielon, MD, Dipartimento Medico Chirurgico di Cardiologia Pediatrica, Ospedale Pediatrico Bambino Gesù, P.zza S.Onofrio 4, 00165 Roma, Italy. Phone: 06-6859-2333; Fax: 06-6859-2257; E-mail: guido.michielon{at}tin.it

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.

Key Words: transplantation • surgical aspects • pulmonary circulation • heart disease • congenital