Cellular and Molecular Pathological Mechanisms in the Failing Heart

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The cardiomyopathies are a group of diseases that affect the heart muscle itself and are not the result of hypertension or congenital or acquired valvular, coronary, or pericardial abnormalities. The term cardiomyopathy should be restricted to a condition primarily involving the myocardium…

Introduction

In many cases, however, it is not possible to arrive at a specific etiologic diagnosis, and thus it is often more desirable to classify the cardiomyopathies into one of three types dilated, restrictive, hypertrophic on the basis of differences in their pathophysiology and clinical presentation. About one in three cases of congestive heart failure (CHF) is due to dilated cardiomyopathy, with the remainder the consequence of coronary artery disease. Left and/or right ventricular systolic pump function is impaired, leading to progressive cardiac enlargement and hypertrophy, a process called remodeling. Symptoms of CHF typically appear only after remodeling has been ongoing for some time. There is, however, no close correlation between the degree of contractile dysfunction and the severity of symptoms. Hypertrophic cardiomyopathy (HCM) is characterized by left ventricular hypertrophy, typically of a nondilated chamber, without obvious cause such as hypertension or aortic stenosis. Two features of HCM have attracted the greatest attention: (1) asymmetric left ventricular hypertrophy, often with preferential hypertrophy of the interventricular septum; and (2) a dynamic left ventricular outflow tract pressure gradient, related to a narrowing of the subaortic area as a consequence of the midsystolic apposition of the anterior mitral valve leaflet against the hypertrophied septum. ...
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