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Congenit Heart Dis. 2016 Dec;11(6):756-765. doi: 10.1111/chd.12392. Epub 2016 Jul 14.

Characteristics of Congenital Coronary Artery Fistulas Complicated with Infective Endocarditis: Analysis of 25 Reported Cases.

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Department of Cardiology, Hospital Group Twente, Hengelo, The Netherlands.


Congenital coronary artery fistulas (CAFs) are infrequent congenital coronary artery anomalies. Complications such as left-to-right shunt, congestive heart failure, myocardial infarction, pericardial effusion, aneurysm formation, rupture, hemopericardium, pulmonary hypertension, infective endocarditis (IE), syncope, stroke, and sudden death may occur with a variable low frequency. To describe the clinical characteristics of patients with CAFs complicated by IE. A search was conducted through PubMed using the terms "CAFs" and "IE." Papers with a full description of the fistula characteristics and detailed data regarding bacterial endocarditis were included for evaluation. In the overall group of reviewed subjects (n = 25, 9 females), the mean patient age was 42.5 years (range: 16 and 87). The right coronary artery (RCA) and left coronary artery (LCA) contributed equally to fistula formation. Terminations into the right heart side occurred in 19 (76%) fistulas. The majority of the fistulas (92%) were unilateral. The cultured microorganism was Streptococcus in 14 (56%) and Staphylococcus in 4 (16%) of the reviewed subjects. Echocardiographic single or multiple valvular regurgitation was found in 8 (32%) of the reviewed subjects. Small and large intracardiac vegetations were detected in 18 patients (72%). Antibiotic therapy was initiated in 20 (80%) subjects and 16 fistulas were treated surgically. During surgery, spontaneous closure of the fistula was observed in one patient. Percutaneous therapeutic embolization (PTE) was successfully performed in two subjects. CAFs complicated by IE may affect all age groups with a slight male preponderance. Unilateral fistulas, either arising from the right or left coronary artery, are predominant, draining mainly into the right heart side. It is emphasized that antibiotic prophylaxis is strongly advised for pediatric and adult patients with congenital CAFs.


Congenital Anomaly; Congenital Coronary Artery Fistulas; Fistula Characteristics; Infective Endocarditis; Surgical Closure; Valvular Heart Disease

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