A rare variant of a ruptured sinus of valsalva aneurysm forming an aorto-atrial fistula: a case report and review of literature

BACKGROUND: Sinus of valsalva aneurysms (SOVAs) are infrequent findings and generally diagnosed incidentally. A SOVA may be at risk for rupture, which would lead to an aorto-cardiac shunt. These patients present similarly to decompensated heart failure. CASE PRESENTATION: We present a case of a 44-year-old female with a ruptured non-coronary SOVA diagnosed by echocardiogram during evaluation for exertional dyspnoea. A trans-oesophageal echocardiogram (TEE) revealed a 2.1 cm non-coronary SOVA with windsock communication to the right atrium. The patient refused surgery, and two years later, presented with florid right heart failure with preserved left ventricular function. The right ventricle was severely dilated and hypokinetic with right atrial enlargement. After finally agreeing to surgery, a pre-operative catheterization revealed non-obstructive coronaries and a significant left to right shunt with elevated pulmonary pressure. The patient had suboptimal response to diuretic therapy and was sent for successful repair of the aneurysm with the closure of the aorto-atrial fistula via bovine pericardial patch and resolution of the left to right shunt as demonstrated by intra-operative TEE. Her right-sided heart failure symptoms subsequently resolved. DISCUSSION: SOVA is a rare finding but should still be considered in the differential in young and middle-aged patients with symptoms of acute heart failure, hemodynamic compromise, and a new continuous heart murmur. Early surgical repair is highly recommended to prevent acute and long-term complications.