One and one half ventricle repairs.

Patients with functionally or anatomically borderline pulmonary ventricles can be managed by a biventricular repair, a Fontan procedure, or by an intracardiac repair in association with a cavopulmonary anastomosis. The latter repair is known as a one and one half ventricle repair. Extending the limits of a biventricular repair can be associated with a high early mortality. The Fontan is associated with late failure. One and one half ventricle repairs are an attempt to reduce early risk and late failure while still achieving separate pulmonary and systemic circulations. The two most common reasons for a one and one half ventricle repair are a small pulmonary ventricle and a dilated poorly functioning pulmonary ventricle. Estimated ventricular volumes of 30% to 80% and z values as small as -10 have been successfully repaired with the one and one half ventricle approach. Ebstein's anomaly is the most common situation where dilated and poorly functioning pulmonary ventricles are treated with a one and one half ventricle repair. There are also special situations where the one and one half ventricle repair is logical. Mortality has ranged from 0% to 12%. No intermediate term sequelae of protein loosing enteropathy, atrial arrhythmias, or pulmonary arteriovenous fistula have been identified. Successful one and one half ventricle repairs have primarily been performed for small pulmonary ventricles and poorly functioning pulmonary ventricles such as those seen with Ebstein's anomaly. Intermediate term follow up has been favorable when compared to the Fontan circulation. Copyright 2000 by W.B. Saunders Company