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Background:  Coronary artery ectasia (CAE) complicated with concomitant congenital coronary artery fistula (CCAF) is rare. This study characterizes the clinical characteristics of CAE combining CCAF, and reports a single-institution experience with surgical correction of CAE combining CCAF.  Methods:  A total of 24 symptomatic patients (8 males, median 52.5 years old) who underwent surgical correction of CAE combining CCAF in this center were reviewed. Based on the size of ectatic segment, the CAE were classified as a giant CAE (&gt;20 mm, n = 14) and a non-giant CAE (≤20 mm, n = 10). Individualized surgical approaches were chosen. The patients were followed up for a median of 3.8 years.  Results:  The overwhelming majority of CAEs were solitary, and only 4.2% of CAEs were associated with multiple lesions. CAEs were predominantly located in the right coronary artery with predilection to women more than to men (2:1). 95.8% of patients with the CCAF had single fistula defect. The right atrium was the most frequent drainage site (33.3%) followed by the left ventricle (25.0%). Surgical mortality was 4.2%. All 22 follow-up patients survived with recovery from symptoms and New York Heart Association (NYHA) functional class I-II. In 10 patients with non-giant CAEs undergoing closure of fistula alone, favorable in-hospital outcomes were recorded, but residual fistula (one patient) and acute inferior wall myocardial infarction related to intracoronary thrombosis (one patient) were observed at follow-up. In 11 patients with giant CAEs undergoing aneurysm resection plus distal bypass grafting at the time of closure of fistula, favorable in-hospital outcomes and encouraging midterm results were recorded. Additionally, in 3 patients with giant CAEs undergoing closure of fistula plus aneurysmal plication, adverse events occurred, including surgical death related to rupture of the ectatic segment (one patient), perioperative myocardial infarction caused by acute thromboembolism (one patient), nonfatal inferior wall myocardial infarction related to intracoronary thrombosis (one patient) at follow-up.Conclusion: Individualized surgical approaches based on the size and the location of ectatic coronary artery as well as fistula should be offered to symptomatic patients with CAE combining CCAF.

Surgical Correction of Coronary Artery Ectasia Combining Congenital Coronary Artery Fistula