Evolution of staged approach for Fontan operation.

Background: During the early development of the Fontan operation, a number of physiologic and anatomical limits were proposed as selection criteria, and two criteria, pulmonary vascular resistance and ventricular function, have been important in predicting surgical outcome. The use of the bidirectional cavo pulmonary shunt as a staging procedure performed to control the pulmonary blood flow adequately and reduce ventricular volume over load has resulted in marked improvements in the early and late Fontan procedure results.
Methods and Results: At our hospital we perform systemic pulmonary shunt or pulmonary artery banding in patients if pulmonary blood flow can not be controlled adequately in the neonatal period and then perform bidirectional cavo pulmonary shunt six months afterwards. During this operation we also performed simultaneous surgical repair for pulmonary artery distortion, anomalies of pulmonary venous connection, restriction of bulboventricular foramen and atrioventricular valve regurgitation. To determine the efficacy of this staged approach in avoiding increases in pulmonary vascular resistance and impaired ventricular function, surgical results were investigated. From February 1995 to May 2001, eighteen patients with cardiac morphology unsuitable for biventricular repair were admitted to our hospital. Twenty-six palliative procedures, were performed including seven pulmonary artery banding, three systemic pulmonary shunt, thirteen bidirectional cavo pulmonary shunt, one original Glenn procedure, four repair of coarctation of the aorta, two total anomalous pulmonary venous connection repair, one mitral valve plasty, and two patients required Damus-Kaye-Stansel procedure to release restrictive bulboventricular foramen. Fifteen patients underwent a modified Fontan operation (total cavopulmonary connection) after these palliative procedures. The operative mortality rate for these palliative procedures was 3.8% (1/26). The operative mortality rate for Fontan operation was 7.1% (1/14). Three patients awaiting the Fontan operation were considered good candidates for a final operation and no patients in this series were considered unsuitable for Fontan completion.
Conclusion: Our strategy of staged approach for Fontan procedure offers a good prognosis.