Arrhythmogenic cardiomyopathy

Domenico Corrado, Cristina Basso, Daniel P. Judge

Research output: Contribution to journalArticle

Abstract

Arrhythmogenic cardiomyopathy is an inherited heart muscle disorder, predisposing to sudden cardiac death, particularly in young patients and athletes. Pathological features include loss of myocytes and fibrofatty replacement of right ventricular myocardium; biventricular involvement is often observed. It is a cell-to-cell junction cardiomyopathy, typically caused by genetically determined abnormalities of cardiac desmosomes, which leads to detachment of myocytes and alteration of intracellular signal transduction. The diagnosis of arrhythmogenic cardiomyopathy does not rely on a single gold standard test but is achieved using a scoring system, which encompasses familial and genetic factors, ECG abnormalities, arrhythmias, and structural/functional ventricular alterations. The main goal of treatment is the prevention of sudden cardiac death. Implantable cardioverter defibrillator is the only proven lifesaving therapy; however, it is associated with significant morbidity because of device-related complications and inappropriate implantable cardioverter defibrillator interventions. Selection of patients who are the best candidates for implantable cardioverter defibrillator implantation is one of the most challenging issues in the clinical management.

Original languageEnglish (US)
Pages (from-to)785-802
Number of pages18
JournalCirculation Research
Volume121
Issue number7
DOIs
StatePublished - Sep 1 2017

Keywords

  • Arrhythmias
  • Arrhythmogenic right ventricular cardiomyopathy
  • Cardiac
  • Cardiomyopathies
  • Defibrillators
  • Desmosomes
  • Implantable

ASJC Scopus subject areas

  • Physiology
  • Cardiology and Cardiovascular Medicine

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  • Cite this

    Corrado, D., Basso, C., & Judge, D. P. (2017). Arrhythmogenic cardiomyopathy. Circulation Research, 121(7), 785-802. https://doi.org/10.1161/CIRCRESAHA.117.309345