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A coronary artery fistula (CAF) is a rare congenital anomaly first reported by Krause in 1865. It is defined as a direct communication between the coronary artery and any surrounding cardiac chamber or vascular structure, which bypasses the myocardial capillary bed. The incidence of small CAFs in an adult population, undergoing cardiac catheterization at the Cleveland Clinic, was 0.13%. In the same series, the incidence of large or multiple fistulas was less than that of small fistulas and was present in 0.05% of all patients screened. The natural history of CAF in adults remains undefined, as does the best approach to managing these patients with recommendations for early closure as well as conservative management found in the medical literature. We present a patient initially diagnosed with a clinically silent CAF who presents 10 years later with symptoms and many of the classic findings of a CAF. An alternative model for the management of CAF in adults is discussed.

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