Newly Discovered Heart Murmur
Noncoronary Sinus of Valsalva Aneurysm With Rupture Into the Right Atrium and Right Ventricle
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. Physical examination revealed a continuous 2/6 systolic-diastolic precordial murmur but was otherwise unremarkable. Transthoracic echocardiography demonstrated a left-to-right shunt that originated from the aortic bulbus, with a slightly enlarged right atrium and ventricle. Transesophageal echocardiography 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 acquired 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 resonance 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 occasionally 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.
The online-only Data Supplement is available with this article at http://circ.ahajournals.org/cgi/content/full/119/3/e15/DC1.