Poor Left Ventricular Performance in a Child With Coronary Sinus Stenosis: Successful Surgical Repair

Atresia or stenosis of the coronary sinus is a rare congenital defect that can cause marked clinical manifestations, depending on the presence of concomitant anomalies securing adequate coronary venous outflow. We describe a 4.5-year-old boy admitted to the clinic with signs of congestive heart failure (left ventricular ejection fraction, 29%). His condition had deteriorated during the previous 4 months. The examination revealed stenosis of the coronary sinus ostium, coronary sinus-to-left atrium fistula, mitral regurgitation of the second grade, and moderate pulmonary hypertension. The surgical intervention consisted of enlargement and plastic reconstruction of the coronary sinus as well as closure of the coronary sinus-to-left atrium fistula. The hospital period was uneventful. The left ventricular ejection fraction at discharge increased up to 50%.