Ruptured sinus of Valsalva aneurysm with associated ventricular septal defect: a case report

Sinus of Valsalva aneurysm (SOVA) is a rare cardiac anomaly, occurring in approximately 0.09% of the population and accounting for 0.1-3.5% of congenital heart defects. It represents a dilation between the aortic annulus and the sinotubular ridge. Rupture occurs in nearly 35% of cases, most commonly from the right coronary sinus into the right heart chambers, and may precipitate acute left-to-right shunting, heart failure, or sudden death. Ventricular septal defects (VSDs) coexist in 30-50% of SOVA cases, further increasing structural and hemodynamic complexity.

We report a 64-year-old woman with no prior cardiac diagnosis who presented with acute dyspnea and a 2-year history of exertional breathlessness. Clinical evaluation revealed a systolic murmur, dilated left ventricle (LVEDD: 59 mm), severe left atrial enlargement (volume index: 61 ml/m²), and moderate pulmonary hypertension (PASP: 48 mm Hg). Echocardiography confirmed a reduced ejection fraction (35%), a membranous VSD, and rupture of the right SOVA into the right ventricle. She underwent successful surgical repair with double patch closure of both the ruptured SOVA and VSD while preserving the aortic valve.

This case highlights the diagnostic and therapeutic challenges of ruptured SOVA with concurrent VSD, especially in older patients. The coexistence of lesions exacerbates shunting and accelerates decompensation. Echocardiography with Doppler flow is crucial for diagnosis. Surgical repair remains the definitive treatment, providing excellent prognosis when performed promptly. The case also underscores the embryological link between membranous VSD and right SOVA, with a higher incidence of right-sided ruptures into the RV observed in Asian populations. Timely recognition, surgical expertise, and valve-preserving techniques are essential for favorable outcomes.

Ruptured SOVA with concurrent VSD is a rare but life-threatening anomaly. Early diagnosis and prompt surgical intervention are critical to optimize survival and preserve cardiac function.