Coronary Artery Fistula Pathophysiology and Management

Coronary artery fistulae are anomalous vascular communications between one or more coronary arteries and a cardiac chamber or great vessel. They may be congenital or acquired and range from small, clinically silent shunts to large, haemodynamically significant connections that provoke myocardial ischaemia, volume overload and heart failure. Pathophysiologically, high‐flow fistulae divert blood away from the myocardial capillary bed (the “coronary steal” phenomenon), causing regional hypoperfusion and compensatory coronary dilation. Over time, turbulent flow can precipitate thrombosis, aneurysm formation and endocardial damage with risk of arrhythmia or endocarditis. Small fistulae often remain asymptomatic in early life, whereas larger defects manifest in adulthood with angina, dyspnoea or exercise intolerance. Management strategies are guided by fistula size, shunt volume, symptomatology and anatomy. Small, asymptomatic fistulae may be managed conservatively with clinical surveillance, while medium to large shunts generally require intervention. Transcatheter closure techniques have become first‐line in many centres, utilising coils, vascular plugs or occluder devices, whereas surgical ligation remains vital for complex anatomies or failed percutaneous procedures. Multimodality imaging—including echocardiography, computed tomography angiography and invasive angiography—underpins accurate anatomical delineation and procedural planning. A multidisciplinary approach ensures optimal selection of device, access route and follow‐up, with the ultimate aim of preventing long‐term complications and preserving myocardial perfusion.

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Coronary Artery Fistula Pathophysiology and Management publication trend

The graph below shows the total number of articles in coronary artery fistula pathophysiology and management across all publications each year (not limited to Nature Index journals).

Technical terms

Coronary artery fistula: An abnormal connection between a coronary artery and a cardiac chamber or great vessel, bypassing the myocardial capillary network.

Transcatheter closure: A minimally invasive procedure to occlude fistulae using coils, plugs or occluder devices delivered via vascular access.

Coronary steal phenomenon: Diversion of blood flow through a low-resistance fistula away from the myocardial microcirculation, leading to ischaemia.

Aneurysm: Focal dilation of the fistula vessel wall, prone to thrombosis, rupture or embolism under high-flow conditions.

Myocardial ischaemia: Insufficient oxygen delivery to heart muscle, usually manifesting as chest pain or regional dysfunction.

Takotsubo cardiomyopathy: A stress-induced transient left ventricular dysfunction that may coexist with or be unmasked by coronary fistulae.

References

1. Three-Decade Experience With Management of Coronary Artery Fistulas in Children. Canadian Journal of Cardiology (2024).
2. A Rare Case of Left Anterior Descending Coronary Artery to Pulmonary Trunk Fistula Associated with Takotsubo Cardiomyopathy. Diagnostics (2023).
3. Midterm follow up of transcatheter closure of coronary artery fistula with Nit-Occlud® patent ductus arteriosus coil. BMC Cardiovascular Disorders (2021).
4. Coronary arterial fistulas. Orphanet Journal of Rare Diseases (2006).