The role of the hepatic factor on the improvement of arterial oxygenation after a Kawashima procedure: A case report

Abstract

Background: Anomalies of the systemic venous return to the heart are infrequent, occurring more often in patients with congenital heart disease.

Case report: We present a patient with heterotaxy, univentricular circulation, and hemiazygos return who underwent a Kawashima procedure at 3 years of age. Fourteen years later, she developed increased cyanosis and tiredness. The symptoms clearly improved after an off-pump derivation of the hepatic drainage from the common atrium to the hemiazygos system.

Conclusions: This rare case report addresses the surgical challenges imposed by patients with complex congenital heart disease combined with anomalies in systemic venous return. Although improvement in arterial oxygenation after redirecting hepatic flow to the hemiazygos system in patients previously submitted to a Kawashima procedure has already been reported, the existence of a hepatic factor remains to be recognized.

Keywords: Kawashima procedure; cyanosis; hepatic factor; heterotaxy syndrome.