Total anomalous venous return, unroofed coronary sinus and left superior vena cava: A rare coexisting triumvirate

Total anomalous venous return, unroofed coronary sinus and left superior vena cava: A rare coexisting triumvirate

Omama Asim , Muneer Amanullah , Yumna Khabir
Dow medical college, Karachi, Pakistan; Sindh institute of urology and transplantation, Pakistan

Background: The coexistence of unroofed coronary sinus (CS), persistent left superior vena cava (LSVC) draining into the left atrium (Raghib’s syndrome), and total anomalous pulmonary venous return (TAPVR) is a rare congenital cardiac anomaly. It results in a ‘‘naturally’’ corrected TAPVR and a CS atrial septal defect presenting as a left-to-right shunting lesion.[1] These conditions require a complex diagnostic and management approach.
Case Presentation: A 1 year and 7-month-old female infant was referred for failure to thrive, congestive heart failure and cyanosis. Transthoracic echocardiography revealed a substantial right-to-left shunt through a small patent foramen ovale (PFO), dilation of the right atrium and ventricle, a partially unroofed coronary sinus, LSVC draining into the CS, and cardiac-type TAPVR with all four pulmonary veins draining into the CS. She also had a large secundum atrial septal defect (ASD) with a left-to-right shunt, RV and PA hypertension, and moderate pulmonary hypertension confirmed on catheterization.
Interventions: The patient underwent complex surgical correction involving unroofing of the coronary sinus, rerouting of the LSVC to the right atrial appendage (Left Warden Procedure), and closure of the ASD directing CS and pulmonary veins to the left atrium. Postoperatively, echocardiography showed improved pulmonary venous flow and moderate bi-ventricular dysfunction. She received intensive monitoring in the PICU.
Outcome: Six months post-surgery, the patient demonstrated significant clinical improvement, normal growth, and improved cardiac function on echocardiography, with no residual shunting or vascular obstruction.
Conclusion: This case highlights that early diagnosis, surgical planning, and a multidisciplinary approach are essential for optimal outcomes in rare complicated congenital heart anomalies.

Keywords: Total Anomalous Pulmonary Venous Return, Unroofed coronary sinus, Persistent Left Superior Vena Cava