Myocardial ischemia with multifactorial pathogenesis is frequently noted in hypertrophic cardiomyopathy (HCM). Coronary artery fistulas (CAF) may be found in HCM but their contribution to myocardial ischemia has not been studied. From 1984 to 2022, 43 patients with HCM and CAF (age 6–82 years, mean 58; 58% male; 77% apical variant; 12% obstructive) were reported in medical literature. Single and multiple CAF were reported in 24 (56%) and 19 (44%), respectively. Overall, a total of 70 major epicardial coronary arteries were affected. CAF arose from left main (LMCA) [1 (1%)], left anterior descending (LAD) [35 (50%)], left circumflex (LCx) [13 (19%)], right (RCA) coronary artery [20 (29%)], and either LAD or LCx [1 (1%)]. The receiving site for CAF was left ventricle (LV) [51 (73%)], pulmonary artery (PA) [10 (14%)], right ventricle (RV) [6 (9%)], both PA and RV [1 (1%)], both left atrium (LA) and PA [1 (1%)] and unspecified in 1. Overall, 28 (65%) patients presented with chest pain, and 14 (33%) had objective evidence of myocardial ischemia (troponin release or positive stress test). Medical therapy was offered to 26 (60%) and 2 (5%) underwent percutaneous or surgical interventions. Follow-up was available for 24 (56%) patients, of whom 23 improved symptomatically while one continued to have episodic chest pain. CAF may be present in patients with HCM, particularly the apical phenotype, and may potentially contribute to myocardial ischemia in this disease. Symptomatic improvement is generally expected in most cases after medical therapy or intervention.

Hypertrophic cardiomyopathy, Apical hypertrophic cardiomyopathy, Coronary fistula, Myocardial ischemia