Left superior vena cava draining into the left atrium: Clinical entities, diagnosis and surgical treatment

Abstract

There are two different clinical entities in which a persistent left superior vena cava (LSVC) can drain into the left atrium (LA) in the absence of coronary sinus: heterotaxy syndrome and unroofed coronary sinus syndrome (UCSS). In these cases, biventricular repair can be done by intracardiac rerouting or by disconnection-reconnection to the right atrium (RA) or to the right superior vena cava (RSVC) using extracardiac procedures. In the period from 1998 to 2016, 10 patients with LSVC draining into the LA underwent 11 surgeries for biventricular repair. The present study focuses on heterotaxy syndrome and unroofed coronary sinus syndrome (UCSS), associated cardiomyopathies, the state of the art in their surgical treatment and our results in our sample. Our experience highlights that accurate diagnosis and appropriate selection of surgical technique, considering different characteristics, such as patient anatomy, age, associated cardiomyopathies, etc., are important for patient outcome.