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Hypertrophic cardiomyopathy: Natural history and prognosis

Author
Martin S Maron, MD
Section Editor
William J McKenna, MD
Deputy Editor
Brian C Downey, MD, FACC

INTRODUCTION

Hypertrophic cardiomyopathy (HCM) is a genetically determined heart muscle disease caused by mutations in one of several sarcomere genes which encode components of the contractile apparatus. (See "Hypertrophic cardiomyopathy: Gene mutations and clinical genetic testing".)

HCM is characterized by an enormous diversity in both phenotypic expression and clinical course (figure 1). The location, pattern, and extent of left ventricular hypertrophy are heterogeneous, although the most common location for increased wall thickness is the basal anterior septum in continuity with the anterior free wall. HCM patients can develop one or more of the following morphologic abnormalities:

LV outflow tract obstruction (see "Hypertrophic cardiomyopathy: Morphologic variants and the pathophysiology of left ventricular outflow tract obstruction")

Diastolic dysfunction

Myocardial ischemia

                     

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Literature review current through: Nov 2016. | This topic last updated: Thu May 19 00:00:00 GMT 2016.
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