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:
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:
Sukakibura, S, Konno, S. Congenital aneurysm of the sinus of valsalva associated with ventricular septal defect – Anatomical aspects. American Heart Journal. 1968;75: