Rare congenital coronary anomaly - unroofed coronary sinus

Abstract

In this paper we describe a case of an eight-month-old male infant who was diagnosed with an unroofed coronary sinus (UCS) that unusually presented with desaturation and cyanosis after surgical ventricular sep-tal defect (VSD) closure. The patient was initially diagnosed with congenital heart disease (CHD), perimembranous VSD as-sociated with mild stenosis of pulmonary arteries in early newborn period. Although the patient was regularly monitored by a paediatric cardiologist, and was even hos-pitalized twice due to deterioration of the initially diagnosed disease and the devel-opment of congestive heart failure (CHF) and arrhythmias, the UCS went unnoticed. The disease was unveiled completely after VSD was surgically closed and the patient’s condition deteriorated postoperatively with desaturation and cyanosis in the in-tensive care unit (ICU). On that same day the patient was reoperated on. UCS was closed with a pericardial patch, a persistent left superior vena cava ligated (PLSVC) and the patient successfully recovered. This case is an example that UCS is not only rare but is such an insidious congeni-tal heart disease, often masked with associ-ated heart defects. It should prompt every paediatric cardiologist to perform a careful examination of coronary sinus inflow tract or performance of contrast echocardiogra-phy in every suspicious case.