Redirection of the hepatic venous flow for the treatment of pulmonary arteriovenous malformations after Fontan operation.

A 45-month-old boy who had complex cardiac anomalies with interrupted inferior vena cava and polysplenia underwent extracardiac Fontan operation. He redeveloped deep cyanosis postoperatively. Cardiac catheterization showed a preferential flow of the hepatic venous blood to the right lung and arteriovenous malformations (PAVMs) in the left lung.

Ventricular mechanics in the bidirectional glenn procedure and total cavopulmonary connection.

Background: The time course of ventricular efficiency in Fontan candidates who underwent both the bidirectional Glenn procedure (BDG) and total cavopulmonary connection (TCPC) were analyzed in this study. We previously reported that volume-load reduction of BDG preceding TCPC allowed for any afterload mismatch to be corrected, thereby improving ventricular efficiency after staged TCPC.

Methods: We measured percent normal systemic ventricular end-diastolic volume (%N-EDV), contractility (end-systolic elastance [Ees]), afterload (effective arterial elastance [Ea]), and ventricular efficiency (ventriculoarterial coupling [Ea/Ees]) based on cardiac catheterization data before and after both BDG and staged TCPC in 30 patients.