Familiar hypertrophic cardiomyopathy in association with coarctation of the aorta and coronary artery disease. Importance of family screening. A brief report
Hountis, P.; Argyriou, M.; Hatziveis, K.; Roumpeas, C.; Antonopoulos, N.
Minerva Cardioangiologica 55(4): 517-519
2007
ISSN/ISBN: 0026-4725 PMID: 17653030 Document Number: 606801
Hypertrophic cardiomyopathy (HCM) is a condition of unknown etiology with thickening of the interventricular septum. It is better defined as a genetic disorder of autosomal dominant inheritance with variable left ventricular hypertrophy and symptoms. HCM has been rarely reported in association with other causes of hypertrophy such as hypertension and aortic stenosis. The combination of aortic stenosis or other arterial stenosis such as coronary artery disease with HCM is unusual. We present a case of a middle aged male with an unusual combination of cardiac diseases. Mild coarctation of aorta, mild aortic stenosis in association with familial HCM and the difficulty in diagnosis are the salient features. The acute change on ECG underscores the presence of coronary artery disease coexistent with the above mentioned diagnosis. The importance of family screening in this situation is highlighted. On the other hand, combined forms of left ventricular outflow obstruction are being recognized with increasing frequency. The importance of recognizing multiple levels of obstruction in such patients is emphasized.