Background: Conversion from total cavopulmonary shunt (TCPS) to the Fontan circulation can improve cyanosis in patients with potential risks of development of pulmonary arteriovenous fistula (PAVF).
Methods: Inclusion of the hepatic veins in the pulmonary circulation was employed using an intra-atrial tube graft in 5 patients with left isomerism previously undergoing TCPS. Prior to the conversion, abnormal communication was identified between the azygos vein and either the hepatic or the portal vein in all. PAVF was seen in 3.
Results: All patients survived the procedure. Postoperative catheterization showed 13 +/- 2 mm Hg of superior caval venous pressure, and 2.3 +/- 0.4 L/min/m2 of cardiac index. Pulmonary arteriovenous fistula progressed markedly in the right lung even after the conversion in 2 patients, in whom the hepatic veins had been exclusively diverted to the left lung. Arterial oxygen saturation became below 65%, with exercise capacity reduced, in these 2 patients. The other patients remain asymptomatic.
Conclusions: Total cavopulmonary shunt can be efficiently converted to the Fontan circulation by appropriately redirecting hepatic venous drainage to perfuse both lungs in a balanced fashion.