Late Diagnosis of Unroofed Coronary Sinus Associated with Persistent Left Superior Vena Cava in Patient with Repaired Tetralogy of Fallot: Case Report

TL;DR

A rare heart defect involving an unroofed coronary sinus and a persistent left superior vena cava was diagnosed years after a patient's Tetralogy of Fallot repair and successfully treated.

Contribution

This case report highlights the rare coexistence of unroofed coronary sinus and Tetralogy of Fallot, emphasizing the importance of late diagnosis and imaging in repaired patients.

Findings

A 20-year-old woman with repaired TOF was found to have a type I unroofed coronary sinus and PLSVC.

The anomaly caused significant right-sided chamber dilation and pulmonary hypertension.

Successful surgical correction was achieved with interatrial compartmentation and tricuspid annuloplasty.

Abstract

Background: An unroofed coronary sinus (UCS) is a rare congenital cardiac anomaly, accounting for less than 1% of atrial septal defects and frequently associated with a persistent left superior vena cava (PLSVC). Its coexistence with Tetralogy of Fallot (TOF) is exceptionally uncommon and has been reported almost exclusively in isolated case reports. Case Presentation: We report the case of a 20-year-old woman with a history of surgically corrected TOF in infancy, who presented with progressive exertional dyspnea. Multimodality imaging, including transthoracic echocardiography and cardiac magnetic resonance imaging, revealed a large atrial-level shunt caused by a type I unroofed coronary sinus associated with a persistent left superior vena cava, leading to significant right-sided chamber dilation and pulmonary hypertension. Notably, this anomaly had not been identified at the time of the initial TOF repair. The patient underwent a successful surgical correction with interatrial compartmentation and tricuspid annuloplasty, with an uneventful postoperative course. Conclusions: This case underscores the extreme rarity of the UCS–TOF association and highlights the potential for UCSs with PLSVC to remain clinically silent for years after complex congenital heart surgery. A comprehensive reassessment of the systemic venous and atrial anatomy using advanced multimodality imaging is essential in symptomatic adults with repaired TOF, as late-presenting venous anomalies may have significant hemodynamic and clinical implications.

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