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Characteristics of Congenital Coronary Artery Fistulas Complicated with Infective Endocarditis: Analysis of 25
1Department of Cardiology, Hospital Group Twente, Hengelo, The Netherlands.
Insights
Congenital coronary artery fistulas (CAFs) complicated by infective endocarditis (IE) predominantly affect adults, often arising unilaterally from the RCA or LCA and draining into the right heart. Antibiotic prophylaxis is crucial for patients with CAFs.
Area of Science:
- Cardiology
- Infectious Diseases
- Congenital Heart Abnormalities
Background:
- Congenital coronary artery fistulas (CAFs) are rare anomalies with potential serious complications.
- Infective endocarditis (IE) is a rare but severe complication of CAFs.
Purpose of the Study:
- To elucidate the clinical characteristics of patients diagnosed with CAFs complicated by IE.
- To identify common microorganisms and treatment outcomes in this patient cohort.
Main Methods:
- A systematic literature search was performed using PubMed with keywords "CAFs" and "IE."
- Included studies provided detailed fistula and IE data.
- Patient demographics, fistula origin and drainage, causative microorganisms, and treatment strategies were analyzed.
Main Results:
- The study reviewed 25 patients (64% male), with a mean age of 42.5 years.
- Right coronary artery (RCA) and left coronary artery (LCA) fistulas were equally common, predominantly unilateral and draining into the right heart (76%).
- Streptococcus (56%) and Staphylococcus (16%) were the most common pathogens; 72% had vegetations, and 32% had valvular regurgitation.
Conclusions:
- CAFs with IE affect all age groups, with a slight male predominance.
- Unilateral fistulas draining to the right heart are most common.
- Antibiotic prophylaxis is strongly recommended for all patients with CAFs.
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.

