The Hidden Dangers of Additional Shunts in Patent Foramen Ovale Treatment

Unroofed coronary sinus (UCS) is a rare congenital defect that causes a left-to-right shunt, often associated with anomalies such as persistent left superior vena cava. Its diagnosis and management are challenging.

A 49-year-old woman with worsening dyspnea, orthopnea, and peripheral edema unresponsive to diuretics was referred for evaluation. She had a history of epilepsy and had had a patent foramen ovale (PFO) closure 1 year before. Transthoracic echocardiography revealed right ventricular dilation, preserved biventricular function, and increased pulmonary pressure. Imaging and right heart catheterization confirmed UCS and partial anomalous pulmonary venous connection (PAPVC). The coronary sinus roof was reconstructed, and a modified Warden procedure corrected the PAPVC.

This case highlights the rare coexistence of PFO, UCS, and dual PAPVC, emphasizing the need for comprehensive diagnostic evaluation and tailored surgical intervention for complex congenital defects.