Case presentation

Coronary artery fistulas are rare vascular anomalies between a coronary artery and a cardiac chamber or great vessel, bypassing the myocardial capillary bed. In children, these are often diagnosed incidentally. Some may require closure, particularly if large or hemodynamically significant. When anatomically feasible, percutaneous closure provides a less invasive alternative to surgery. ^{Reference Al-Hijji, Sabbagh and Hajj1}

The authors report the case of a previously healthy 7-year-old boy who was referred due to a heart murmur. On examination, a continuous murmur was evident. The patient reported no chest pain, palpitations, or syncope. Diastolic blood pressure was mildly reduced, with normal systolic pressure. The electrocardiogram was unremarkable, showing no signs of ventricular ischaemia; however, transthoracic echocardiography revealed a large 7 mm coronary fistula arising from the left main coronary artery, which was markedly dilated, draining into the apex of the right ventricle. Both ventricles were mildly dilated, but systolic function was preserved, and no significant mitral or tricuspid regurgitation was observed.

Selective left coronary angiography confirmed a large fistula with a maximal diameter of 8 mm and a distal stenotic segment of 4 mm (Figure 1a-c). Given the large diameter and high flow rate, an Amplatzer™ Vascular Plug II was selected for its capacity to adapt to variable vessel sizes, allow controlled deployment, and reduce the risk of embolisation. Closure was achieved via an aortic access, using an arteriovenous loop (Figure 1d). A 12 × 9 mm plug was successfully deployed via anterogradely (Figure 1e), with post-procedural angiography demonstrating complete occlusion of the fistula, absence of flow within the blind-ended pouch, and improved perfusion of the left anterior descending and circumflex arteries (Figure 1f). The patient was discharged on low-dose aspirin (5 mg/kg/day) for 6 months to reduce thrombotic risk within the residual blind pouch. Follow-up echocardiography showed no device protrusion into the right ventricle, and at 3 months confirmed normalisation of ventricular size and preserved biventricular systolic function, with no evidence of thrombus or residual shunt. Electrocardiogram remained without signs of ischaemia.

Figure 1.

Fluoroscopic images demonstrating percutaneous closure of a large coronary artery fistula in a 7-year-old boy using an arteriovenous loop: ( a ) Left anterior oblique (LAO)-cranial projection shows, on an unselective aortic root angiogram, a markedly dilated left main coronary artery giving rise to a fistulous tract; ( b ) LAO-caudal projection, selective left coronary angiography demonstrates a large, tortuous fistula draining into the right ventricle; ( c ) Right anterior oblique (RAO)-caudal projection provides enhanced visualisation of the fistula’s tortuosity and more clearly delineates the drainage site into the right ventricle; ( d ) LAO-cranial projection shows establishment of an arteriovenous loop via snaring in the pulmonary trunk; ( e ) LAO projection, deployment of a 12 × 9 mm Amplatzer™ Vascular Plug II in the distal fistulous segment; ( f ) LAO-caudal final angiography demonstrates complete occlusion of the fistula, absence of flow within the blind-ended pouch, and improved perfusion of the left anterior descending and circumflex arteries.

This case highlights the importance of percutaneous closure as an effective alternative to surgery for the treatment of large coronary artery fistulas in paediatric patients.