A rare case of unruptured extracardiac multiple sinus of Valsalva aneurysms originating from the orifices with partial aortic wall defects

Background: Sinus of Valsalva aneurysm (SVA) is relatively rare and commonly reported as a congenital anomaly. It is usually found in a single Valsalva sinus protruding into another cardiac chamber and is termed as intracardiac SVA. The aneurysm usually originates from the Valsalva sinus itself, and an orifice of the aneurysm is observed surrounded by the aortic wall. Thus, extracardiac multiple SVAs originating from the orifices with partial aortic wall defects are extremely rare. We report a very rare case of unruptured extracardiac SVAs in both left and right coronary sinuses originating from the aortic annulus.

Case presentation: A 76-year-old Japanese male was diagnosed with enlarged Valsalva sinuses by transthoracic echocardiography during follow-up for peripheral artery disease. Five years after careful observation, gradual SVA enlargement and moderate aortic insufficiency were observed. He underwent modified Bentall's procedure, with an uneventful postoperative course. Intraoperatively, SVAs were found in the left lateral half of the left and right coronary sinuses of Valsalva on both sides of the commissure between the left and right coronary cusps. Aortic walls were missing at the SVA floor adjacent to the aortic annulus. Pathological examination revealed only mild atherosclerotic changes of the aortic wall near the SVAs. The cause was estimated as either focal degeneration of the sinuses of Valsalva just above the aortic annulus or congenital anomaly, or combination of both of them.

Conclusions: We report on the case of unruptured extracardiac multiple SVAs missing aortic orifice just above the annulus. No similar case presentation was found in the literature. In this paper, we present details of operative findings and procedures, which will aid in procedure selection.