Transesophageal Echocardiographic Assessment of Single Ventricle Physiology with Interrupted Inferior Vena Cava in a Case of Heterotaxy Syndrome Posted for Kawashima Procedure and Hepatic Vein Rerouting

Abstract

Transesophageal echocardiography (TEE) is essential in delineating the anatomy, physiology, and perioperative management of single ventricle (SV) lesions. SV lesion is commonly associated with heterotaxy syndrome causing abnormal lateralization of organs across the body’s left-right axis. It is manifested as right atrial or left atrial isomerism. Patients with SV physiology require the Glenn procedure for surgical palliation whereas Fontan operation as definitive intervention. However, in a patient with interrupted inferior vena cava (IVC), the Kawashima procedure is used for definitive palliation. We report a 15-year-old female child diagnosed with SV physiology and interrupted IVC in association with heterotaxy syndrome posted for the Kawashima procedure and hepatic vein rerouting and illustrate the importance of perioperative TEE in delineating the anatomy, decision-making, and confirming the success of repair.