Congenital circumflex artery-coronary sinus fistula in an adult female associated with severe mitral regurgitation and myelodysplasy--case report and review of the literature.

BACKGROUND

Coronary artery fistulas are uncommon anomalies. They occur in 0.1-0.2% of patients undergoing coronary arteriography. The origin of the fistulas is the right coronary artery followed by the left anterior descending and lastly by the circumflex artery (17%). Termination into the right heart side occurs in 90% of cases. Termination into the coronary sinus is rare in 3% of cases. Circumflex artery-coronary sinus fistulas are even rarer.

DESIGN

A single case report and literature review between 1993 and 2007.

RESULTS

We describe a 76-year-old female, who was analyzed for dyspnea on exertion (DOE) and chronic fatigue, with known myelodysplastic syndrome and an aneurysmal circumflex coronary artery-coronary sinus fistulous connection associated with severe mitral regurgitation. Mitral valve replacement using a bioprosthesis was performed as well as ligation of the fistula. The postoperative course was complicated with cardiac tamponade, which was successfully drained.

CONCLUSION

Our patient presented with chronic fatigue and DOE and was found to have a coronary artery fistula and severe mitral regurgitation associated with known myelodysplasia. Conventional coronary angiography failed to demonstrate the entire fistula characteristics (origin, pathway, and outflow). Multidetector computed tomography was complementary to demonstrate the complex anatomy of the fistula. The fistula was surgically ligated in combination with mitral valve replacement. She remains well.