Case report: desmoplakin cardiomyopathy presenting as an inflammatory cardiomyopathy with repeated sudden cardiac

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Emmanuelle Massie1, Arnaud Dominati2, Sebastian Suchet3

  • 1Cardiology Service, University Hospital of Geneva, Rue Gabrielle Perret-Gentil 4, 1205 Geneva, Switzerland.

European Heart Journal. Case Reports
|June 18, 2024
PubMed

Insights

Desmoplakin cardiomyopathy, a rare heart condition, presents with inflammation and scarring. This case highlights a new gene variant and discusses potential, though limited, benefits of immunosuppressive treatment for this challenging diagnosis.

Area of Science:

  • Cardiology
  • Genetics
  • Molecular Biology

Background:

  • Desmoplakin cardiomyopathy is a recently classified non-dilated left ventricular cardiomyopathy.
  • It involves inflammatory episodes, left ventricular fibrosis, dysfunction, and ventricular arrhythmias.
  • Current management strategies remain unclear.
Abstract

Keywords:
ArrhythmiaCardiomyopathyCase reportDesmoplakinInflammationSudden cardiac death

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