1349960 - Bicuspid Aortic Valve and Ruptured Sinus of Valsalva Aneurysm: Role of Cardiovascular Magnetic Resonance Imaging

Description of Clinical Presentation: 33 year old male presented with an asymptomatic murmur. Bicuspid aortic valve (BAV) and mild aortic regurgitation was diagnosed on transthoracic echocardiography (TTE). Follow up TTE, a year later revealed a small tricuspid valve mass which was investigated further with transesophageal echocardiography (TEE) that showed findings suspicious for ventricular septal defect (VSD). He was referred to our adult congenital heart disease (ACHD) clinic for further evaluation. Repeat TEE at our university hospital demonstrated findings of ruptured sinus of Valsalva (SOV). Blood cultures and TEE showed no conclusive findings of endocarditis. Cardiovascular magnetic resonance imaging (CMR) was performed to confirm diagnosis, preoperative planning to determine accurate aortic size and rule out complex congenital heart disease. Patient was referred for surgical repair after the CMR.

Diagnostic Techniques and Their Most Important Findings: BAV is commonly associated with aortopathies and TTE is an excellent initial technique to identify and follow-up patients with BAV. However, TTE may be technically limited for accurate aortic sizing in BAV and in cases of asymmetric root enlargement (1, 2). In our case, although a left to right shunt was identified on an earlier TTE, initial impression was a high velocity VSD jet. TEE helped identify ruptured sinus of Valsalva along with TTE and doppler findings. CMR confirmed the diagnosis and helped evaluate the entire length of thoracic aorta and quantifiable ventricular enlargement, which was missed on previous TTE; an important parameter in considering surgical repair in asymptomatic patients. We obtained cine right ventricular outflow, cine short axis and reduced field of views of aorta (3 slices) to define the anatomical relationship of BAV and sinus of Valsalva aneurysm (SOVA) to the adjacent structures. Cine left ventricular outflow stacks were used to distinguish high VSD from SOVA. Phase contrast of aorta was used to quantify flows and regurgitation. CMR confirmed the ruptured SOVA at non coronary cusp and demonstrated continuous left to right shunt into right atrium with Qp:Qs of 1.6 by phase contrast imaging as well as severe left ventricle dilation and mild biventricular systolic dysfunction. BAV was fused at right and non-coronary cusps without stenosis and with trivial regurgitation. TTE and TEE underestimated the SOV at 3.9 cm; however, MRA revealed the SOVA at 4.2 cm. Furthermore, CMR demonstrated that tricuspid valve mass was a part of ruptured SOVA. Findings are described in Figure 1 and 2.

Learning Points from this Case: Rupture of SOVA involving the non-coronary cusp is rare. TTE and TEE may be sub optimal in defining the full extent of sinus of Valsalva aneurysm and complications. CMR provides excellent temporal resolution, quantification of flows, shunt, volumes and function allowing for identification of aneurysm and complications in addition to relationship to the adjacent structures facilitating timely clinical decision making as well as perioperative planning and management.