EDUCATION IN HEART

Pathology

New insights into the pathology of inherited cardiomyopathy

Siân E Hughes¹, William J McKenna²

¹ Department of Histopathology, Royal Free and University College Medical School, University College London, London, UK
² Department of Cardiology, The Heart Hospital, UCL Hospitals NHS Trust, London, UK

Correspondence to:
Correspondence to:
Dr Siân E Hughes
Department of Histopathology, Royal Free and University College Medical School, University College London, UCL Hospitals NHS Trust, Rockefeller Building, University Street, London WC1E 6JJ, UK; sian.hughes@ucl.ac.uk

Keywords: cardiomyopathy; endomyocardial biopsy; sudden death

The first 150 words of the full text of this article appear below.

Cardiomyopathies are defined as diseases of the myocardium, which cause cardiac dysfunction with heart failure, arrhythmia, and sudden death. Cardiomyopathies represent a major cause of morbidity and mortality in both children and adults and are a frequent indication for cardiac transplantation. In 1995, the World Health Organization (WHO)/International Society and Federation of Cardiology (ISFC) task force recommended that the cardiomyopathies be classified into two main groups: specific cardiomyopathies, and primary cardiomyopathies.¹ The specific cardiomyopathies include heart muscle disease associated with myocarditis, specific cardiac disease or general systemic disease. In contrast, the primary cardiomyopathies are diseases intrinsic to the myocardium itself and are classified pathophysiologically. This group includes dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), restrictive cardiomyopathy (RCM), arrhythmogenic right ventricular cardiomyopathy (ARVC), and unclassified cardiomyopathy.

DILATED CARDIOMYOPATHY AND ISOLATED LEFT VENTRICULAR NON-COMPACTION

Idiopathic dilated cardiomyopathy (DCM) is the most common cause of congestive heart failure in the young with an estimated prevalence of at least 36.5 per . . . [Full text of this article]

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