Presentation 18 year old male with no medical history was admitted with syncope

1. Sinus of Valsalva Fistula Without Aneursym Mark A. Navarro M.D., Joseph R. Sweigart M.D., Becky E. Selling, Lucy B. Esberg M.D., Edward Havranek M.D., Whitney Juselius M.D. • Presentation • 18 year old male with no medical history was admitted with syncope • Frequent episodes of lightheadedness lasting around 1 minute for the past 1 month • 2 episodes daily at the time of presentation • No pain, diaphoresis, nausea, vomiting, or loss of bladder or bowel control • He took no medications, never smoked, rarely drank, and never used drugs • No known family history of cardiac disease LA LA Ao LV o Ao LV Fistula IVS RV RV • Physical Exam and Data • Temperature 36.5° Celsius, 72 beats per minute, 118/47 mmHg, 14 breaths per minute, oxygen saturation 96 percent on room air • Continuous machinery murmur audible throughout the precordium with palpable thrill along the left sternal border • Exam otherwise normal with normal labs • Chest X-ray showed cardiomegaly • EKG with RSR’ in V1 and V2 • Transthoracic echocardiogram showed membranous ventricular septal defect (VSD) but no explanation for the machinery murmur • Trans-esophageal echocardiogram showed VSD as well as sinus of valsalva (SoV) fistula between the aorta to right ventricle • Hospital Course • Decision made to pursue surgical correction • Catheterization documented pulmonary to systemic blood flow ratio of 2.2:1 • Operation revealed small VSD and fistula near the right coronary ostium separated by a small annulus adjacent to the right coronary cusp • VSD was repaired with synthetic material • SoV fistula was repaired but with subsequent aortic insufficiency requiring placement of mechanical aortic valve • No aneurysmal dilation of SoV visualized • Uncomplicated peri-operative course • Discussion • Fistulous connection between the aorta and the right ventricle without aneurysm is quite rare • SoV fistula is most often a congenital defect and part of a spectrum of anomalies including SoV aneurysm • 2/3 of SoV aneurysms occur in males and up to 80% occur in the right coronary sinus • Most common symptoms are dyspnea, fatigability and chest pain. • The most common congenital anomaly associated with SoV aneurysm is VSD which is present in 12-53% of cases • Conclusions • SoV fistulization is likely part of a spectrum of congenital anomalies including SoV aneurysm and often coinciding with VSD • Unexpected symptoms and exam findings should be investigated thoroughly until an explanation is discovered • This is a rare case of sinus of valsalva fistula without evidence of aneursymal formation. • References • Moustafa, S, Mookadam, F, Copper, L et al. Sinus of Valsalva Aneurysms – 47 Years of a Single Center Experience and Systematic Overview of Published Reports. The American Journal of Cardiology. 2007;99:1159-1164. • Lee, S, Lin, T, Chiu, C, et al. Ruptured Aneurysm of the Sinus of Valsalva into the Right Atrium without Ventricular Septal Defect: A Case Report and Literature Review. Kaohsiung J Med Sci. 2005;21:517-521. • Sukakibura, S, Konno, S. Congenital aneurysm of the sinus of valsalva associated with ventricular septal defect – Anatomical aspects. American Heart Journal. 1968;75:595-603.