Abstract

Introduction: Arteriovenous coronary fistula of LAD to the coronary sinus is a rare vascular anomaly present in 0.002% of the general population. We report a rare case of fistulous connection between exceptionally large LAD and coronary sinus into the LV cavity leading to severe systolic heart failure. Case: A 39-year-old male with no past medical history presented to the ED with complaints of dyspnea, chest pain and lower extremity edema for the past 3 months. The chest pain was substernal, squeezing, non-radiating, 2/10 in intensity, exacerbated by exertion and alleviated with rest. The patient did not have any cardiology work up in the past. Vitals were stable. The patient had irregular rhythm with prominent JVD and bilateral lower extremity edema on examination. Troponins were negative, BNP was elevated and thyroid function was normal. Chest x-ray showed cardiomegaly. EKG showed atrial fibrillation with no significant ST segment changes or Q waves. Echocardiogram showed severe systolic left ventricular dysfunction with an ejection fraction of 15-20% and moderate to severe secondary mitral regurgitation. Cardiac catheterization showed large ectatic LAD with mild stenosis in mid segment. An abnormal connection was noted between LAD and coronary sinus draining directly into the left ventricle. He was discharged on guideline directed medical therapy for heart failure. Discussion: Fistula between LAD, coronary sinus and LV cavity is a unique organic heart disease. Our patient presented with heart failure with no apparent identifiable cause. On angiography an ectatic LAD was connected to the coronary sinus and LV cavity which created a high output state on the LV. The management of this rare pathology is complex due to variable presentation and lack of specific treatment guidelines. Careful selection of certain cases for therapeutic intervention can result in better outcomes than medical therapy alone.

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