Revision of previous Fontan connections to total extracardiac cavopulmonary anastomosis: A multicenter experience

Background: Conversion to total extracardiac cavopulmonary anastomosis is a n option for managing patients with dysfunction of a prior Fontan connectio n, Methods: Thirty-one patients (19.9 +/- 8.8 years) underwent: revision of a previous Fontan connection to total extracardiac cavopulmonary anastomos is at four institutions, Complications of the previous Fontan connection in cluded 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 enteropath y (n = 3), right atrial thrombus (n = 2), subaortic stenosis (n = 1), atrio ventricular valve regurgitation (ri = 3), and Fontan baffle leak (n = 5), C onversion 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 death s. Two patients died in the perioperative period of heart failure and massi ve 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. E arly postoperative arrhythmias occurred in 10 patients: 4 required pacemake rs, and medical therapy was sufficient in 6, In 15 patients, pre-revision a rrhythmias were improved, Effusions resolved in all but 1 of the patients i n 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 ca n be achieved with low morbidity and mortality, Optimally, revision should be undertaken early in symptomatic patients before irreversible ventricular failure ensues.