Myocardial ischemia and congestive heart failure from a left main to coronary sinus fistula
Ranjan Dahiya1, Jack Copeland, Samuel M Butman
1Section of Cardiology, The University of Arizona, Tucson, Arizona, USA.
Insights
Coronary artery fistulas (CAFs) are rare congenital heart defects. This case highlights a silent CAF developing symptoms over a decade, prompting a discussion on adult CAF management strategies.
Area of Science:
- Cardiology
- Congenital Heart Disease
- Vascular Surgery
Background:
- Coronary artery fistula (CAF) is a rare congenital anomaly defined as a direct communication between a coronary artery and a cardiac chamber or vascular structure.
- The incidence of CAFs in adults undergoing cardiac catheterization is low, with small CAFs at 0.13% and large/multiple CAFs at 0.05%.
Observation:
- The natural history and optimal management of CAFs in adults are not well-defined.
- A patient initially diagnosed with a clinically silent CAF presented 10 years later with symptomatic disease and classic CAF findings.
Findings:
- This case illustrates the potential for a clinically silent CAF to progress and become symptomatic over time.
- The presentation of delayed symptoms underscores the need for careful monitoring and consideration of long-term outcomes.
Implications:
- The findings suggest that a reassessment of management strategies for adult CAFs may be warranted.
- An alternative model for managing adult CAFs, considering potential long-term progression, is proposed.
Abstract:
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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