CONGRESS ABSTRACT

A 35-year-old male patient presented to the emergency department with recurrent episodes of palpitations. Transthoracic echocardiography (TTE) revealed eccentric left ventricular hypertrophy with mild systolic dysfunction and a suspected ventricular septal defect (VSD) with bidirectional shunt flow. The aortic valve was bicuspid, non-stenotic, and non-regurgitant, while the right ventricle was dilated but showed preserved systolic function. Transesophageal echocardiography (TEE) further identified aneurysmal dilation of the right coronary sinus with a fissure, resulting in a left-to-right shunt into the right atrium. Additionally, a single coronary artery was noted. Contrast-enhanced CT angiography and 3D coronary CT reconstruction confirmed a right-sided aorto-atrial/ventricular fistula with a 17 mm communication near the tricuspid valve and an ectatic right coronary sinus, corroborating the left-to-right shunt. Coronary circulation was left-dominant, with a high origin of the left main coronary artery and absence of the right coronary artery, replaced by a small branch arising from the mid-left anterior descending artery. The patient underwent a successful aortotomy with closure of the perforation in the anterior coronary sinus using continuous sutures. Intraoperative TEE confirmed the absence of residual shunt and revealed mild aortic valve regurgitation. At 3- and 12-month follow-ups, TTE showed normalized cardiac dimensions and function. The bicuspid aortic valve (BAV) demonstrated moderate regurgitation, while right ventricular and atrial dimensions and function remained within normal ranges. One-year follow-up scintigraphy was negative for ischemia, and genetic sequencing revealed no mutations linking the BAV, the right aorto-atrial/ventricular fistula, and the single coronary artery. The BAV and single coronary artery findings warrant regular follow-up to monitor for potential future complications, including coronary artery disease and progressive valve dysfunction.