Chronic rupture of the coronary sinus of Valsalva with pseudoaneurysm formation

A 45-year-old male with no significant medical history or risk factors for coronary disease presented with a 1-year history of intermittent, exertional chest pain radiating to the neck. Physical examination was unremarkable. An EKG was normal. A chest X-ray, however, revealed a 4-cm round focus with peripheral calcification overlying the left side of the heart. A computed tomography (CT) of the chest revealed a spherical area with rim calcification in the area of the aortic root on the left. The differential diagnosis at that point was: a calcified left coronary artery aneurysm, left ventricular anterior wall calcified pseudoaneurysm or a pseudoaneurysm arising from the left aortic root. This prompted a CT angiogram of the coronary arteries. His coronary arteries all appeared normal on imaging, but the calcified mass was localized to have originated from the left coronary sinus of Valsalva. It appeared to be a ruptured aneurysm of the left coronary sinus, which led to the formation of a pseudoaneurysm with peripheral calcification. It was felt that the process was chronic due to the long history of angina and calcification present within the sac. The superior margin of the neck of the pseudoaneurysm was 2 mm inferior to the origin of the left coronary artery, and the inferior margin was contiguous with the hinge point of the left leaflet (Fig. 1A). His symptoms were thought to be related to external compression of the proximal left main coronary artery (LMCA; Fig. 1B). To better assess the aortic root and coronary arteries, the patient underwent cardiac catheterization, which confirmed the finding of normal coronary arteries, but revealed displacement of the LMCA due to mass effect from the pseudoaneurysm (Fig. 1C—left). Selective angiography of the aortic root and left coronary sinus revealed a thick layer of organized thrombus within the sac without fistulization (Fig. 1C—right). It was recommended he undergoes surgical resection and repair to prevent further complications, including hemodynamically significant rupture, fistula formation or heart failure, but the patient refused to undergo operative management. True aneurysms of the sinus of Valsalva (ASVs) are either congenital or acquired. Congenital ASVs are rare anomalies most often caused by the absence of muscular and elastic tissue in the aortic wall behind the sinus of Valsalva. Acquired ASVs are caused by conditions that affect the integrity of the aortic wall such as infections (e.g. bacterial endocarditis, syphilitic aortitis or tuberculosis), degenerative disease (e.g. atherosclerosis and connective tissue disease) or trauma. Most ASVs originate from the right coronary sinus (94%), and a minority originates from the non-coronary sinus (5%). Very rarely is the left coronary sinus affected [1, 2]. Pseudoaneurysms—or false aneurysms—are formed when a break in the intimal layer of the sinus causes blood to collect within a false lumen covered by the outer adventitia. They are more likely to occur due to localized intimal disease or trauma during procedures or surgeries. In many cases, a true aneurysm is present before the tear occurs [3, 4]. Obstruction of coronary flow secondary to aneurysms or pseudoaneurysms of the sinus of Valsalva is an uncommon manifestation. The risk of flow disturbance is substantially greater when the left coronary sinus is involved causing compression of the LMCA, left anterior descending and/or left circumflex artery [3, 5, 6].