The association of mitral valve disease and coronary artery fistula (CAF) is a rarely seen antity. In most of the cases, CAF is eitherasymptomatic or represents angina-like symptoms. However, the symptoms and physical examination findings may be masked whenit is associated with a valvular disease. In this study, we report a CAF case associated with mitral valve stenosis. Aconnection betweenright coronary artery and right ventricle was detected by coronary angiographic study performed before valve replacement surgery onthe patient who had only dyspnea, fatigue and palpitation complaints. During the surgical procedure, which included mitral valvereplacement by using prosthesis, fistula site was found and ligated. Among the case presentations that represent coronary arteryanomalies associated with mitral valve disease, there is no any fistula opening into right ventricle. In the very limited cases thatpresented as CAF associated with valvular disease (mitral and/or aortic), the fistula usuallyopens into either pulmonary artery or rightatrium.

Keywords : Mitral valve stenosis, coronary artery fistula, open heart surgery, right ventricle