Abstract
Myocardial ischemia is a frequent finding in hypertrophic cardiomyopathy (HCM) potentially resulting from microvascular dysfunction, extravascular compression, reduced coronary flow reserve, hemodynamic abnormalities, or concomitant atherosclerotic coronary artery disease (CAD). From June 1991 to October 2024, 40 congenital coronary artery anomalies (CCAA) were reported in 30 adults with HCM [age 18–87 (47.6 ± 19.7); 60% male; 37% obstructive; 20% apical variant]. Clinical data on ischemia, therapy, and outcomes were reviewed. Coronary anatomy was categorized by number of ostia: single (n=12; 40%), two (n=13; 43%), three (n=4; 13%), or four (n=1; 4%). The anomalous vessel was the left main (20%), left anterior descending (25%), left circumflex (18%), right coronary artery (35%), or ramus intermedius (2%). Two patients (7%) with apical HCM also had coronary fistulae. Myocardial ischemia was present in 13 patients (43%), including 5 with anomalous vessels coursing between the great vessels. Two of five patients with sudden cardiac arrest had a high-risk anomaly. Pharmacologic therapy was provided to 13 (43%), coronary surgery to 3 (10%), and septal reduction to 8 (27%). Among 15 patients with follow-up, all reported symptomatic improvement. CCAA may occur in HCM and contribute to ischemia; management should be guided by anomaly type and objective evidence of ischemia.
Keywords
Hypertrophic cardiomyopathy, HCM, Coronary artery, Coronary anomalies, Myocardial ischemia