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

Abstract

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.

Coronary Artery Fistulas: Pathophysiology, Imaging Findings, and Management

“…Valvular regurgitation due to papillary muscle abnormality also has been reported in patients with CAFs (21). The estimated prevalence of infective endocarditis in these patients is 3%-12% (22). Therefore, after patent flow through the CAF is detected, prophylaxis for endocarditis is recommended (9,13,17).…”

Fistula between the right coronary artery and coronary sinus: a case report and literature review

“…The possibility of natural closure of a congenital coronary artery fistula is very small. For patients with other congenital heart diseases, surgical treatment should be particularly performed as early as possible to avoid some related complications (9, 10). …”

Recommendations for Multimodality Assessment of Congenital Coronary Anomalies: A Guide from the American Society of Echocardiography

“…A large CAF can cause significant complications, including congestive heart failure due to the large shunt through the fistulous connection, myocardial ischemia related to steal of CA blood flow from the CAF, thrombus formation in the dilated proximal CA segment, ventricular arrhythmias, infectious endocarditis, pulmonary hypertension, syncope, stroke, and even sudden death. 86 American College of Cardiology/American Heart Association guidelines recommend closure of all large CAFs regardless of symptomatology; smaller than moderate sized CAFs with symptoms; or CAFs associated with documented myocardial ischemia, arrhythmia, otherwise unexplained ventricular systolic/diastolic dysfunction, ventricular enlargement, or endarteritis. 87 Either transcatheter device closure or surgical ligation to obliterate the shunt without compromising the normal CA branches is utilized when intervention is needed.…”