Unroofed coronary sinus and persistent left superior vena cava: A case report

Abstract

The unroofed coronary sinus is a spectrum of cardiac anomalies in which part or all of the common wall between the coronary sinus and the left atrium is absent. Most cases are associated with anomalous systemic venous return, such as persistent left superior vena cava.The diagnosis of this lesion is important to the prognosis of the patient, but it is often difficult because of nonspecific clinical features.Here we report a case of a complete unroofed coronary sinus and persistent left superior vena cava discovered during a computed tomographic investigation for pulmonary hypertension.

Persistent Left Superior Vena Cava in Cardiac Congenital Surgery

“…Reports in the literature concerning persistent LSVC are centered on difficulties, in patient without congenital heart disease, in placing central venous catheter or pacemaker lead insertions or related complications [12,15,17,22]. In contrast, there are many reports of persistent LSVC associated with rare combination of congenital heart disease or reports on persistent LSVC and associated anomalies of the coronary sinus [2,3,21]. The global impact of persistent LSVC has, to our knowledge, never been evaluated during the perioperative and postoperative period of cardiac surgery for congenital heart disease in children.…”

Transcatheter occlusion of a persistent left superior vena cava to the left atrium using the transseptal approach

“…In patients with congenital heart defects, a persistent LSVC is seen in up to 10% of patients; additional cardiac defects include atrial septal defect, bicuspid aortic valve, coarctation of aorta, coronary sinus ostial atresia, cor triatriatum, tetralogy of Fallot and heterotaxy syndrome . Persistence of the LSVC is due to failed involution of the left anterior and left common cardinal veins during the 7th gestational week . The diagnosis of a persistent LSVC is usually an incidental finding, suggested by the presence of a dilated coronary sinus on echocardiogram, or during placement of a central venous catheter from the left subclavian or jugular vein.…”

“…However, in 8% of patients with a persistent LSVC, the LSVC drains to the left atrium through an unroofed coronary sinus . An unroofed coronary sinus has four morphologic classifications; type I, which was present in our patient, is a “completely unroofed” coronary sinus with the persistent LSVC draining directly into the left atrium . This venous anomaly causes a right‐to‐left shunt, as desaturated systemic venous blood directly enters the left atrium.…”

“…Persistence of a LSVC is a relatively common anatomical variant, with an incidence of 0.3% in autopsy studies . In patients with congenital heart defects, a persistent LSVC is seen in up to 10% of patients; additional cardiac defects include atrial septal defect, bicuspid aortic valve, coarctation of aorta, coronary sinus ostial atresia, cor triatriatum, tetralogy of Fallot and heterotaxy syndrome . Persistence of the LSVC is due to failed involution of the left anterior and left common cardinal veins during the 7th gestational week .…”

Diagnosis of Persistent Left Superior Vena Cava: Lessons Learned from a Recurrent Stroke Case

“…Previous reports have suggested that up to 75% to 83% of unroofed coronary sinus cases are associated with PLSVC. 2,[7][8][9][10][11][12] A majority of PLSVC cases (80%-90%) involve the PLSVC draining to the right atrium through a dilated coronary sinus and are typically asymptomatic. 1,2,5 However, when the PLSVC communicates with the left atrium, either directly or via the unroofed coronary sinus or pulmonary vein, a right-to-left shunt results.…”