The role of the Fontan procedure in the surgical treatment of congenital heart malformations with double-outlet right ventricle.

The Fontan procedure in one of its many modifications is applicable to the surgical treatment of those patients with double-outlet right ventricle who are not amenable to bi-ventricular correction. Included in this group are patients with significant hypoplasia of one of the ventricles, patients with distant (non-committed) ventricular septal defect, patients with significant straddling of the atrioventricular valves, patients with abnormal atrioventricular connections (such as absence of one of the valves), or common atrioventricular valve connection. The presence of complex associated lesions such as those seen in the heterotaxia syndromes may, by virtue of the surgical risk of attempts at bi-ventricular repair, make the Fontan approach a suitable option for these patients. The presence of a subaortic conus and the potential for subaortic obstruction, a frequent findings in patients with double outlet right ventricle, plays an important role in the preparation and conduct of the Fontan operation. Copyright 2000 by W.B. Saunders Company