Embolization of a Large Right-Coronary-Artery-to-Left-Atrium Fistula in a Three-Year-Old Child: A Case Report.

Objectives: Coronary artery fistulas (CAFs) are rare congenital anomalies with an occurrence rate of 0.002-0.3%. The right coronary artery (RCA) is reportedly the most common site of origin of CAFs, but fistulas draining to the left atrium (LA) are rare. We presented a three-year-old boy with a symptomatic congenital RCA-to-LA fistula, which was successfully percutaneously occluded with an Amplatzer vascular plug 4 (AVP4).

Case report: The diagnosis was made by echocardiography when he was two months old. During the follow-up period of 2 years, a progressive dilatation of the RCA and enlargement of the left ventricle was detected, so treatment for congestive heart failure was initiated. At the age of three, the patient presented with a history of occasional mild central chest pain and discomfort and mild dyspnea on exertion. On a 24 h ECG Holter monitor, the depression of ST segments was registered. CT angiography highlighted a large type B RCA fistula to the LA, which extended along the atrioventricular sulcus. The proximal RCA diameter was 7 mm. The fistula was tortuous, with segmental narrowing and three curves. Cardiac catheterization was performed across the right femoral artery on the three-year-old boy (body weight: 13 kg). Across the 4F Judkins right guiding catheter, an AVP4 of 5 mm was placed in the distal part of the CAF connected with the delivery cable. After 15 min, ECG changes were not registered, so the device was released. Immediate post-deployment angiography demonstrated complete CAF occlusion, with satisfying flow in the distal coronary artery. The patient was discharged after four days. In the short-term follow-up period, the boy was symptom-free.

Conclusions: In our experience, given the existence of the left-to-left shunt and the more pronounced exercise-induced coronary steal phenomenon that occurs in medium-sized and large CAFs, occlusion is necessary to prevent the further progression of clinical signs and symptoms.