Protocols associated with no mortality in 100 consecutive Fontan procedures.

OBJECTIVES Results of Fontan's procedure have improved considerably, but perioperative mortality still occurs, attributed to ventricular dysfunction, stroke, arrhythmia, thromboembolism, and multi-organ dysfunction. Our protocols of operative and intensive care unit management address these potential issues, and have been associated with zero mortality, even with many high-risk candidates. METHODS From 1996 to 2006, all Fontan patients were managed as follows: operative strategy based on aortic and single atrial cannulation, cooling on full-flow bypass, and hypothermic circulatory arrest to create the Fontan pathway. No direct caval cannulation. Use of central venous lines was completely avoided. Fresh whole blood was used for pump prime and for volume restoration. Inotropic and vasodilator therapy was continued for at least 48 h. Aspirin was used exclusively as anti-thrombotic therapy. Postoperative pleural drainage was accomplished with small pigtail catheters. The usual Fontan pathway was by lateral atrial tunnel (84), with extra-cardiac conduit when dictated by anatomy (16). RESULTS One hundred Fontan operations were performed with no mortality. All patients were extubated by postoperative day 1. Hospital stay was 10+/-5 days. Complications were: bleeding (1), reintubation (1), emergent fenestration closure (1), pericardial effusion (4), and seizures (1). Risk factors included Fontan connection to one lung (3), diminutive pulmonary arteries (PAs) and unifocalized major aortopulmonary collateral arteries (MAPCAs) (1), discontinuous PAs (3), right ventricle dependent coronaries (3), neonatal pulmonary venous obstruction (3), Trisomy 21 (1), preoperative pacemaker dependence (2), and heterotaxy (10). No candidate was excluded. CONCLUSIONS While many surgeons try to avoid bypass or aortic clamping when performing Fontan operations, the strategies we have employed facilitate safe accomplishment of Fontan's operation in diverse anatomic groups with multiple risk factors, with avoidance of operative mortality in 100 consecutive cases.