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

AbstractBackground: 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. (J Thorac Cardiovasc Surg 2000;119:340-6

Implantation of a Berlin Heart as single ventricle by-pass on Fontan circulation in univentricular heart failure

The clinical management of ventricular failure after the Fontan operation presents a formidable challenge to surgeons. We report our experience with successful implantation of a Berlin Heart EXCOR ventricular assist device as a bridge to transplantation in a child with Fontan circulation

Bidirectional Glenn and antegrade pulmonary blood flow: temporary or definitive palliation?

BACKGROUND: We sought to investigate the role of the bidirectional Glenn with antegrade pulmonary blood flow in the surgical history of children with univentricular hearts. METHODS: A series of 246 patients, from three joint institutions, having univentricular heart with restricted but not critical pulmonary blood flow received a bidirectional cavopulmonary shunt with additional forward pulmonary blood flow. All patients have been studied according to their progression, or not, to Fontan operation. Two hundred and eight (84.5%) patients underwent bidirectional cavopulmonary anastomosis as primary palliation. Twenty patients (8.1%) with previous pulmonary artery banding were also enrolled in the study. Patients who had received additional pulmonary blood flow through a previous systemic to pulmonary artery shunt for the critical pulmonary blood flow were excluded. RESULTS: No in-hospital death occurred. Follow-up was complete at 100%. Mean follow-up was 4.2 +/- 2.8 years (range, 6 months to 7 years). During the observational period 73 (29.7%) patients, considered optimal candidates, underwent Fontan completion for increasing cyanosis and (or) hematocrit and (or) fatigue with exertion. Three patients expired after total cavopulmonary connection (3 of 73; 4.1% mortality rate). The remaining 173 (70.3%) patients are alive with initial palliation. All patients were still well palliated with an arterial oxygen saturation at rest about 90%. CONCLUSIONS: According to our experience and results, bidirectional Glenn with antegrade pulmonary blood flow may be an excellent temporary palliation prior to a Fontan operation, which can be performed at the onset of symptoms. Bidirectional Glenn may also be the best possible palliation for a suboptimal candidate for Fontan