Diagnostic and Surgical Challenges in Atypical Right Coronary Sinus-Right Atrial Rupture With Concurrent VSD.

Background

Sinus of Valsalva aneurysm is a rare anomaly that, when ruptured, causes high-flow shunts and rapid-onset heart failure. We report a rare anatomical variant of right coronary sinus aneurysm rupturing into the right atrium with a concurrent perimembranous ventricular septal defect (pmVSD). CASE SUMMARY A 16-year-old male with known pmVSD presented with progressive NYHA functional class III dyspnea for 15 days. Volume overload was initially misattributed to ventricular septal defect progression. However, transthoracic echocardiography demonstrated a restrictive pmVSD (3.4 mm opening, peak gradient 60 mm Hg), elevated right ventricular systolic pressure (76 mm Hg) as a flow-mediated surrogate for pulmonary artery systolic pressure, and a ruptured sinus of Valsalva aneurysm with a high-velocity jet into the right atrium. Because of "washout" artifacts, coronary computed tomography angiography failed to visualize the fistulous tract. Single-stage surgical repair of both lesions achieved complete recovery.

Discussion

This case emphasizes color Doppler echocardiography's value in dual-shunt physiology when coronary computed tomography angiography is inconclusive, supporting single-stage surgical repair for multilesion presentations.