Abstract 1623: Coronary Angiodysplasia Associated With Hypertrophic Cardiomyopathy

Background :Coronary Angiodysplasia (CA) is a rare congenital anomaly. Its prevalence as well as histological and clinical implications in Hypertrophic Cardiomyopathy (HCM) are unknown. Methods and results: Among 945 patients diagnosed at our Institution as having HCM, 395 had a coronary angiography and 136 a left ventricular endomyocardial biopsy to rule out ischemic or storage myocardial diseases. Six of 395 (1.2%) (4 M /2 F, mean age 48±14 ys, MWT=29±4mm) presented diffuse coronary teleangectases draining into the left ventricular cavity (Panel A and B), compared with 1 on 1000 (0,1%) of consecutive coronary angiographies of patients with no congenital heart disease. Clinical profile and histology of endomyocardial tissue from the 6 HCM-CA patients was correlated with those of 22 HCM pts matched for age, sex and severity of LV hypertrophy. Histology showed in HCM-CA patients a more pronounced fibrosis of subendocardial layer (30±7 vs 5±2 of HCM) and disorganized cardiomyocytes separated by thin-walled ...