"... . any classification is necessarily incomplete and acts as a bridge between complete ignorance and total understanding...." (Goodwin JF: The frontiers of cardiomyopathy, Br Heart J 1982;48:1-18) nhe conditions we now call cardiomyopathies were regarded as a diversity of "uncommon noncoronary myocardial diseases" in the 1950s.' Goodwin and colleagues2 defined cardiomyopathies as "4primary h...

Disorders of the Pericardium Pericardial Effusion Pericarditis Acute Pericarditis Chronic Pericarditis With Effusion Constrictive Pericarditis Coronary Heart Disease Coronary Circulation Pathogenesis of Coronary Heart Disease Chronic Ischemic Heart Disease Stable Angina Variant Angina Silent Myocardial Ischemia Acute Coronary Syndromes Unstable Angina/Non–ST-Segment Elevation Myocardial Infarct...

Cardiovascular diseases and, among them, channelopathies and cardiomyopathies are a major cause of death worldwide. The molecular and genetic defects underlying these cardiac disorders are complex, leading to a large range of structural and functional heart phenotypes. Identification of molecular and functional mechanisms disrupted by mutations causing channelopathies and cardiomyopathies is es...

Andrew Maslow MD Department of Anesthesiology Rhode Island Hospital, Providence Rhode Island Introduction Cardiomyopathy is generally defined as a “disease of the myocardium associated with cardiac dysfunction”. 1 Primary cardiomyopathies are divided into three major classifications: a) dilated cardiomyopathy (DCM), b) hypertrophic cardiomyopathy (HCM), and c) restrictive (or infiltrative) card...

arrhythmogenic right ventricular dysplasia/cardiomyopathy (arvd/c) is a congenital cardiac disease with myocardial involvement, most probably right ventricular (rv) dysfunction, accounting for 20% of sudden cardiac deaths. characterized by the fibro-fatty infiltration of the rv free wall, arvd/c presents in adolescents with ventricular arrhythmias and heart failure symptoms and as biventricular...

Cardiac magnetic resonance imaging (CMR) has undergone considerable technology advances in recent years, so that it is now entering into mainstream cardiac imaging practice. In particular, CMR is proving to be a valuable imaging tool in the detection, morphological assessment and functional assessment of cardiomyopathies. Although our understanding of this broad group of heart disorders continu...

From the Department of Genetics,* Harvard Medical School, Boston; the Cardiovascular Division,y Brigham and Women’s Hospital and Harvard Medical School, Boston; the Laboratory for Molecular Medicine,z Partners HealthCare Center for Personalized Genetic Medicine, Boston; and the Department of Pathology,x Massachusetts General Hospital, Brigham and Women’s Hospital and Harvard Medical School, Bos...

Copyright © 2011 Massachusetts Medical Society. I nherited cardiomyopathies are a major cause of heart disease in all age groups, often with an onset in adolescence or early adult life. Not only the patients but also their families can be severely burdened by these illnesses. More than 20 years ago, the first “disease gene” for hypertrophic cardiomyopathy was identified.1,2 This finding led to ...

The fact that different types of cardiomyopathies can be manifested by the same sarcomere protein gene mutation in a single family is well known. However, mixed features of different types of cardiomyopathies in a single patient have not been well appreciated. We identified a novel mutation in cardiac troponin I3 (Arg186Gly) in the present case, and two of the family members showed mixed morpho...

Cardiomyopathies are chronic diseases of heart muscle, in which the muscle is abnormally enlarged, thickened, and/or stiffened (1). According to American Heart Association, “Cardiomyopathies are a heterogeneous group of diseases of the myocardium associated with mechanical and/or electrical dysfunction that usually (but not invariably) exhibit inappropriate ventricular hypertrophy or dilatation...