Newly Discovered Heart Murmur Noncoronary Sinus of Valsalva Aneurysm With Rupture Into the Right Atrium and Right Ventricle Tobias Hoevelborn, MD; Joerg Doering, MD; Stephan Lindemann, MD; Christian S. Haas, MD A 37-year-old man was referred for evaluation of a heart murmur detected recently during a routine checkup. The patient had an unsuspicious medical history, without previous infection, trauma, or connective tissue disease. He was completely asymptomatic while resting or exercising. Physi- cal examination revealed a continuous 2/6 systolic-diastolic precordial murmur but was otherwise unremarkable. Trans- thoracic echocardiography demonstrated a left-to-right shunt that originated from the aortic bulbus, with a slightly enlarged right atrium and ventricle. Transesophageal echocardiogra- phy confirmed a noncoronary sinus of Valsalva aneurysm with rupture into the right atrium (Figure, A). Doppler imaging studies showed a prominent jet into the right atrium (flow velocity 4.5 m/s) and a smaller jet into the right ventricle (Figure, B; online-only Data Supplement Movie I). Of note, the obtained ECG was unsuspicious (Figure, C). Magnetic resonance imaging demonstrated that the rupture site was located on the right side posterior to the tricuspid septal leaflet and communicated with the right atrium and ventricle (Figure, D, E, and F), which resulted in a left-to- right shunt with a shunt volume of 20%, as assessed by the phase-contrast method. Cardiac catheterization with bulbus angiography 4 weeks later confirmed the findings of a ruptured sinus of Valsalva aneurysm into the right atrium and ventricle (Figure, G and H; online-only Data Supplement Movie II), revealing an increased left-to-right shunt volume of 50%. The patient was scheduled for urgent surgical repair of the ruptured aneurysm. Sinus of Valsalva aneurysms are rare congenital or ac- quired cardiac anomalies, most commonly involving the right or noncoronary sinuses. 1 Congenital sinuses of Valsalva aneurysm account for 0.5% to 3% of all congenital cardiac defects. In addition, inflammatory diseases, such as syphilis, tuberculosis, endocarditis, and Behçet disease, as well as traumatic events and connective tissue disorders, such as Marfan syndrome, can be the cause of this anomaly. 2 Rupture of a sinus of Valsalva aneurysm requires definitive repair because of progressive right heart failure. Magnetic reso- nance imaging with the phase-contrast method is a reliable noninvasive way to assess the cardiac shunt ratio, thereby helping to guide therapy. 3 The treatment of choice is a surgical approach, 4 although transcatheter occlusion has oc- casionally been shown to be an alternative. 5 Sudden onset of a cardiac murmur should always include the differential diagnosis of a ruptured sinus of Valsalva aneurysm. We here report a very rare case of a ruptured sinus of Valsalva aneurysm into both the right atrium and ventricle, which resulted in an increased left-to-right shunt with the need for urgent definitive repair. Disclosures None. References 1. Ott DA. Aneurysm of the sinus of Valsalva. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2006;1:165–176. 2. Feldmann DN, Roman MJ. Aneurysms of the sinuses of Valsalva. Car- diology. 2006;106:73– 81. 3. Gatehouse PD, Keegan J, Crowe LA, Masood S, Mohiaddin RH, Kreitner KF, Firmin DN. Applications of phase-contrast flow and velocity imaging in cardiovascular MRI. Eur Radiol. 2005;15:2172–2184. 4. Moustafa S, Mookadam F, Cooper L, Adam G, Zehr K, Stulak J, Holmes D. Sinus of Valsalva aneurysms: 47 years of a single center experience and systematic overview of published reports. Am J Cardiol. 2007;99: 1159 –1164. 5. Chang CW, Chiu SN, Wu ET, Tsai SK, Wu MH, Wang JK. Transcatheter closure of a ruptured sinus of Valsalva aneurysm. Circ J. 2006;70:1043–1047. From the Department of Medicine (T.H., S.L., C.S.H.), Division of Cardiology and Cardiovascular Disease, and Department of Radiology (J.D.), University Hospital Tuebingen, Tuebingen, Germany; and Department of Medicine I (C.S.H.), University of Luebeck, Luebeck, Germany. The online-only Data Supplement is available with this article at http://circ.ahajournals.org/cgi/content/full/119/3/e15/DC1. Correspondence to Christian S. Haas, MD, University of Luebeck, Department of Medicine I, Ratzeburger Allee 160, 23538 Luebeck, Germany. E-mail cs_haas@yahoo.com (Circulation. 2009;119:e15-e16.) © 2009 American Heart Association, Inc. Circulation is available at http://circ.ahajournals.org DOI: 10.1161/CIRCULATIONAHA.108.775619 e15 Images in Cardiovascular Medicine Downloaded from http://ahajournals.org by on October 15, 2021