Unique Pattern of Aortic Regurgitation Caused by Rupture Into the Left Ventricular Interleaflet Triangle of a Sinus Valsalva Aneurysm Involving the Left Coronary Cusp
A 43-year-old man with a primary complaint of prolonged paroxysmal coughing was admitted to our hospital for evaluation of a to-and-fro heart murmur. When he initially visited our hospital, his heart failure symptom was classified as New York Heart Association functional classification III. His upper limb blood pressure was 129/52 mm Hg and, pulse rate was 95 beats/min. He had a normal respiratory rate with an O2 saturation of 98% on room air. Moreover, his lower limb blood pressure was 190/48 mm Hg. Hence, transthoracic echocardiography was performed for suspected severe aortic regurgitation based on physical findings. However, transthoracic echocardiography showed a flattened, saccular aneurysm originating in the right sinus of Valsalva, which enlarged at the septum of the left ventricle and appeared to rupture into the left ventricular outflow tract (Figure, A and B). After this, cardiac computed tomography was performed to examine the structural details of the sinus of Valsalva aneurysm (SVA). Three-dimensional (3D) contrast computed tomography clearly showed the SVA via exterior and intraheart views. The 3D computed tomography scans revealed that the SVA extended saccularly behind the left coronary cusp (LCC) and ruptured at the left ventricular interleaflet triangle between the left and right coronary cusp, involving the LCC itself as well (Figure C-1–C-3; Movies I and II in the Data Supplement). Therefore, 2 different regurgitant flow paths were inferred. Because of the involvement of the LCC in SVA rupture and the shortened leaflet of LCC, severe aortic valve regurgitation (a significant eccentric regurgitant jet at the LCC) was detected near the rupture site of the SVA on real-time 3D transesophageal echocardiography (Figure D-1 and D-2; Movies III and IV in the Data Supplement), which was also visible in the surgeon’s actual view (Figure E). The patient needed to undergo surgical repair for both the rupture of SVA and severe aortic regurgitation to relieve symptoms of heart failure. We first closed SVA rupture during surgery, and then we confirmed that the aortic root appeared healthy with normal tissue and the aortic annulus was not dilated at all. Therefore, aortic valve replacement was safely performed. No other congenital heart diseases were detected, including ventricular septal defects. Histopathologic findings of the excised specimen indicated a mild, nonspecific inflammatory response in the LCC. The patient’s postoperative course was uneventful. Although rupture of the SVA usually occurs at the right coronary sinus progressing into the right chambers of heart, a rare case of the SVA rupture into the left ventricle detected by 2-dimensional echocardiography has been reported previously.1 Park et al2 reported a rare case of an SVA that originated from the LCC that ruptured into the left atrium along with severe aortic regurgitation because of aortic valve prolapse. Consequently, we herein report the only case of severe aortic regurgitation caused by rupture of the SVA beginning at the right coronary cusp, following into the left ventricular outflow tract while penetrating through the LCC, as diagnosed by 3D imaging modalities. It was difficult to accurately diagnose this patient’s condition of heart failure using transthoracic echocardiography alone. This extremely rare pathogenesis of severe aortic regurgitation was appropriately diagnosed using 3D computed tomography and real-time 3D transesophageal echocardiography.
The Data Supplement is available at http://circheartfailure.ahajournals.org/lookup/suppl/doi:10.1161/CIRCHEARTFAILURE.117.004440/-/DC1.
Circ Heart Fail is available at http://circheartfailure.ahajournals.org.
- © 2017 American Heart Association, Inc.