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Carlo F. Marcelletti
Frank L. Hanley
Constantine Mavroudis
Doff B. McElhinney
Stefano M. Marianeschi
Francesco Seddio
Ed Petrossian
Luisa Colagrande
Carl L. Backer
Francis Fontan
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J Thorac Cardiovasc Surg 2000;119:340-346
© 2000 Mosby, Inc.

SURGERY FOR CONGENITAL HEART DISEASE

REVISION OF PREVIOUS FONTAN CONNECTIONS TO TOTAL EXTRACARDIAC CAVOPULMONARY ANASTOMOSIS: A MULTICENTER EXPERIENCE

Carlo F. Marcelletti, MDa, Frank L. Hanley, MDb, Constantine Mavroudis, MDc, Doff B. McElhinney, MDb, Raul F. Abella, MDa, Stefano M. Marianeschi, MDa, Francesco Seddio, MDa, V. Mohan Reddy, MDb, Ed Petrossian, MDb, Teresa de la Torre, MDa, Luisa Colagrande, MDa, Carl L. Backer, MDc, Adriano Cipriani, MDa, Fiore S. Iorio, MDa, Francis Fontan, MDd

From the Hesperia Hospital,a Modena, Italy; University of California,b San Francisco, Calif; Children’s Memorial Hospital,c Chicago, Ill; and Clinique St Augustin,d Bordeaux, France.

Address for reprints: Carlo F. Marcelletti, MD, Pediatric Cardiac Surgery, Hesperia Hospital, Via Arquà 80/A, Modena, 41100 Italy (E-mail: marianeschi{at}hesperia.it).

Background: Conversion to total extracardiac cavopulmonary anastomosis is an option for managing patients with dysfunction of a prior Fontan connection.
Methods: Thirty-one patients (19.9 ± 8.8 years) underwent revision of a previous Fontan connection to total extracardiac cavopulmonary anastomosis at four institutions. Complications of the previous Fontan connection included atrial tachyarrhythmias (n = 20), progressive heart failure (n = 17), Fontan pathway obstruction (n = 10), effusions (n = 10), pulmonary venous obstruction by an enlarged right atrium (n = 6), protein-losing enteropathy (n = 3), right atrial thrombus (n = 2), subaortic stenosis (n = 1), atrioventricular valve regurgitation (n = 3), and Fontan baffle leak (n = 5). Conversion to an extracardiac cavopulmonary connection was performed with a nonvalved conduit from the inferior vena cava to the right pulmonary artery, with additional procedures as necessary.
Results: There have been 3 deaths. Two patients died in the perioperative period of heart failure and massive effusions. The third patient died suddenly 8 months after the operation. All surviving patients were in New York Heart Association class I (n = 20) or II (n = 7), except for 1 patient who underwent heart transplantation. Early postoperative arrhythmias occurred in 10 patients: 4 required pacemakers, and medical therapy was sufficient in 6. In 15 patients, pre-revision arrhythmias were improved. Effusions resolved in all but 1 of the patients in whom they were present before revision. The condition of 2 patients with protein-losing enteropathy improved within 30 days.
Conclusions: Conversion of a failing Fontan connection to extracardiac cavopulmonary connection can be achieved with low morbidity and mortality. Optimally, revision should be undertaken early in symptomatic patients before irreversible ventricular failure ensues.




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