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 vessels adherent to the endocardium (Panel C). On a mean follow-up of 16±2 years in the HCM-CA group 2 patients died and 2 required an ICD implantation because of ventricular tachyarrhythmias while no death and 2 ICD implantations were observed in the HCM group.
Conclusions: CA is 12 times more common in HCM than non-HCM population. Its occurrence carries an increased risk of myocardial fibrosis and sudden death.