• Guido Michielon, Francesco Parisi, Duccio Di Carlo, Cosimo Squitieri, Adriano Carotti, Michaela Buratta, and Roberto M Di Donato.
• Department of Cardiac Surgery, Ospedale Pediatrico Bambino Gesù, Rome, Italy. guido.michielon@tin.it
• Eur J Cardiothorac Surg. 2003 Oct 1;24(4):502-10; discussion 510.

ObjectiveEvaluation of incremental risk factors for early mortality in children undergoing orthotopic heart transplantation (OHT) for failing single ventricle physiology.MethodsBetween 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.ResultsThirty-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.ConclusionsOHT 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.

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