Multimodality delineation of a fistulous ruptured sinus of Valsalva aneurysm: a teaching case report

Abstract

Background: Ruptured sinus of Valsalva (SOV) is a rare cardiac anomaly with poor prognosis if untreated. Early diagnosis with accurate delineation of its anatomy is critical for timely treatment and choice of surgical vs. percutaneous intervention. Here we report a case of fistulous rupture of SOV; the preoperative multimodality studies including echocardiography, cardiac magnetic resonance and cardiac catheterization provided teaching and learning points. Case summary: A 48-year-old man with history of heart murmur and hypertension presented with a 5-day history of shortness of breath and peripheral oedema. He was diagnosed with rapid atrial flutter. The transthoracic and transesophageal echocardiography showed severe biventricular systolic dysfunction with a left-to-right shunt from ruptured SOV. The colour Doppler by transthoracic and transesophageal echocardiography and cardiac magnetic resonance revealed a swaying shunt flow exiting in direction to the right atrium (RA) and basal right ventricle (RV) during systole and diastole with no myocardial scaring. The left and right heart catheterization showed elevated right-sided pressures, pulmonary capillary wedge pressure, and left ventricular end-diastolic pressure. There was no difference in O2 saturation between venae cavae and RA but a misleading step-up in O2 saturation between RA and RV. Owing to rupture anatomy with uncertainty, the patient underwent surgical intervention. The ruptured SOV tunnelled through the base of tricuspid annulus to the RA very close to the basal RV. Discussion: Even with multimodality studies it can still be challenging to delineate the anatomy of a ruptured SOV without uncertainty preoperatively.