Unroofed Coronary Sinus and Persistent Left Superior Vena Cava as a Cause of Complication after Surgical Correction of a Double Outlet Right Ventricle

Abstract

Unroofed Coronary Sinus (URCS) is a rare cardiac malformation, which was first described by Raghib et al. [1] in 1965, characterized by partial or complete fenestration between the coronary sinus and the left atrium. It is frequently associated with the existence of a left superior vena cava (PLSVC) and the clinical outcome is variable, from behaviour which is similar to an interatrial septal defect to the possibility of paradoxical embolism due to a right-left shunt at the level of the left atrium.

It can also be associated to other heart disorders, such as atrioventricular septal defects, interventricular communication, interatrial communication, cortriatriatum, tetralogy of Fallot or partial anomalous venous drainage and, if not diagnosed prior to the main surgical correction, may lead to post-surgical complications.

We present the case of a young infant with Double Outlet Right Ventricle with subaortic ventricular septal defect and side by side great arteries, persistent left superior vena cava and ostium secundum atrial septal defect who underwent surgery to correct the septal defects. Post-surgical evolution was difficult, however she recovered after diagnosis and treatment for an unroofed coronary sinus.