Introduction: Coronary artery fistula (CAF) is a connection between one or more of the coronary arteries and a cardiac chamber or great vessel. This is a rare defect and occurs in only 0.2% of the population. Most of the cases are congenital but acquired causes are also reported. A CAF may occur as an isolated cardiac defect or with other cardiac diseases such as rheumatic heart disease (RHD). Only a few cases of coexisting CAF and RHD have been reported. Local data reports only 0.69% CAFs associated with congenital malformations of the heart. Only 61 patients among all patients who underwent coronary arteriography in 34 years were reported to have a CAFs. We report a case of severe mitral stenosis (MS) with a double CAF from the left circumflex (LCx) artery draining into the left atrium.

Case: A 46-year old female with RHD with severe MS came in due to progressive dyspnea. The coronary angiogram revealed two fistulous tracts originating from the LCx draining into the left atrium. She underwent mitral valve replacement (MVR) surgery, left atrial plication, and closure of the fistula drainage the left atrium. The postoperative course was uneventful.

Discussion: A CAF is often asymptomatic until the second decade of life. Untreated, this may progress and cause ischemic and heart failure signs and symptoms. The presence of MS caused elevated left atrial pressure which might have prevented the increase in the volume of blood draining from the LCx artery to the left atrium through the fistulas. Hence, the MS might have prevented the dilatation of the two fistulas. Surgical correction is also indicated in the fistulas since resolution of the mitral stenosis with MVR will decrease the LA pressure which might result to dilatation and increased drainage of the fistulas causing complications later.

Keywords: Coronary artery fistula, Rheumatic heart disease, Case Report  

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