Early results of the fontan procedure in one hundred consecutive patients with hypoplastic left heart syndrome

OBJECTIVES: The purpose of this study was to review a large, single institutional experience with the Fontan procedure for patients with hypoplastic left heart syndrome. METHODS: One hundred consecutive patients with "classic" hypoplastic left heart syndrome underwent Fontan palliation between February 1992 and April 1998. Patient demographic, morphologic, and procedural variables were examined and analyzed. In particular, two different surgical techniques were used: technique I (February 1992 to December 1995) employed cardiopulmonary bypass and moderate systemic hypothermia, and technique II (December 1995 to April 1998), profound hypothermia and circulatory arrest. A retrospective review of medical records was performed and variables were examined and analyzed. RESULTS: Hospital survival for the entire cohort was 89% (95% CI 83%-95%). The technique of operation, cardiopulmonary bypass time, and aortic crossclamp time were each strongly associated with survival. Survival for patients treated by technique I was 79% (95% CI 68-91%; n = 48) and for those treated by technique II, 98% (95% CI 94%-100%; n = 52). Cardiopulmonary bypass and crossclamp times were also highly correlated with time to extubation and length of intensive care unit stay. Preoperative pulmonary artery pressure was correlated with survival; preoperative oxygen saturation, right atrial pressure, pulmonary vascular resistance, pulmonary artery size, extent of aortopulmonary artery collaterals, and echocardiographic estimates of ventricular function and tricuspid regurgitation were not correlated with survival. CONCLUSIONS: Our recent experience with Fontan palliation for patients with hypoplastic left heart syndrome suggests that it is attended by low perioperative mortality. The precise operative technique used appears to be an important determinant of outcome, with the duration of cardiopulmonary bypass and crossclamping being particularly significant.