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A Journal on Heart and Vascular Diseases

Official Journal of the Italian Society of Angiology and Vascular Pathology
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Minerva Cardioangiologica 2007 August;55(4):517-9


language: English

Familiar hypertrophic cardiomyopathy in association with coarctation of the aorta and coronary artery disease. Importance of family screening. A brief report

Hountis P. 1, Argyriou M. 2, Hatziveis K. 3, Roumpeas C. 4, Antonopoulos N. 2

1 Department of Thoracic and Vascular Surgery Athens Naval and Veterans Hospital, Athens Greece 2 Department of Cardiac Surgery Evangelismos General Hospital, Athens, Greece 3 Laboratory of Pharmaceutical Chemistry Department of Pharmacy University of Patras, Patras, Greece 4 Private Practice, Kalamata, Greece


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.

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