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Guido Michielon
Francesco Parisi
Duccio Di Carlo
Cosimo Squitieri
Adriano Carotti
Roberto M. Di Donato
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Right arrow Congenital - cyanotic
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Eur J Cardiothorac Surg 2003;24:502-510
© 2003 Elsevier Science NL


Orthotopic heart transplantation for failing single ventricle physiology

Guido Michielona*, Francesco Parisib, Duccio Di Carloa, Cosimo Squitieria, Adriano Carottia, Michaela Burattaa, Roberto M. Di Donatoa

a Department of Cardiac Surgery, Ospedale Pediatrico Bambino Gesù, Rome, Italy
b Department of Pediatric Cardiology, Ospedale Pediatrico Bambino Gesù, Rome, Italy

Received 5 November 2002; received in revised form 23 April 2003; accepted 26 April 2003.

* Corresponding author. Tel.: +39-06-6859-2333; fax: +39-06-6859-2257
e-mail: guido.michielon{at}tin.it

Objective: Evaluation of incremental risk factors for early mortality in children undergoing orthotopic heart transplantation (OHT) for 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.

Key Words: Transplantation • Heart disease • Congenital




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