Fontan Operation and 1½ Ventricular Repair

Abstract

The Fontan operation has undergone many revisions since its introduction by Francis Fontan and Guillermo Kreutzer in 1968. Gone are the venous pathway valves, the right atrial connections to small subpulmonic ventricles, and atriopulmonary connections. The standard therapy for orthoterminal (Fontan) correction is determined by three distinct time periods: The first is the neonatal management of pulmonary artery flow (by systemic to pulmonary artery shunt, pulmonary artery band, or observation). The second stage takes place at about 6 months of age; it includes systemic to pulmonary artery shunt takedown or pulmonary artery band takedown and a bidirectional Glenn shunt. This procedure prepares the single ventricle by volume unloading, resulting in favorable ventricular remodeling. At about 2 years of age, the Fontan operation is usually performed. Since the introduction of atrial fenestration, some surgeons have used this obligatory right-to-left shunt in all cases, but some use it selectively and others, not at all. Most surgeons now use the fenestration selectively, especially in cases with borderline ventricular function, small pulmonary arteries, abundant aortopulmonary artery collaterals, and single-lung physiology. The fenestration can be performed easily in the lateral tunnel Fontan operation, but not so easily in the extracardiac Fontan operation.