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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

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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