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Current Cardiology Reports

Desmoplakin Cardiomyopathy: Role of Inflammation and Potential Role of Disease-Modifying Therapies

  • 01-12-2025
  • Myocarditis
  • Myocardial Disease (A Abbate and M Merlo, Section Editors)
Published in:
Authors
Alessio Gasperetti
Richard T. Carrick
Steven Muller
Brittney Murray
Luigi Adamo
Barbara Bauce
Elizabeth McNally
Adam Helms
Published in
Current Cardiology Reports | Issue 1/2025

Abstract

Purpose of Review

To summarize the available data on the use of immunosuppression therapies for the management of hot phases of disease and recurrent myocarditis in patients with desmoplakin cardiomyopathy (DSP-CMP).

Recent Findings

Occurrence of myocarditis episodes has been associated with worsening of outcomes in DSP-CMP. Multiple case reports and small case series have described potential benefit in using anti-inflammatory and immunosuppressive medications for the treatment of those episodes. Recently published translational data has shown a clear link between DSP variants and a propensity to inflammation.

Summary

The presence of acute myocarditis episodes have been clearly demonstrated as a manifestation of DSP-CMP. These episodes are marked by myocardial inflammation and subsequent fibrosis, and, moreover, contribute to a heightened future risk of subsequent arrhythmias and heart failure. Identifying optimal strategies to prevent and/or interrupt myocardial inflammation is therefore a critical priority of patients with DSP-CMP.
Title
Desmoplakin Cardiomyopathy: Role of Inflammation and Potential Role of Disease-Modifying Therapies
Authors
Alessio Gasperetti
Richard T. Carrick
Steven Muller
Brittney Murray
Luigi Adamo
Barbara Bauce
Elizabeth McNally
Adam Helms
Publication date
01-12-2025
Publisher
Springer US
Published in
Current Cardiology Reports / Issue 1/2025
Print ISSN: 1523-3782
Electronic ISSN: 1534-3170
DOI
https://doi.org/10.1007/s11886-024-02183-7
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Independent Medical Education Grant:
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Image Credits
Abstract graphic of layered, concentric circular shapes in bright green, pink, blue, and purple on a dark blue background. The rings and segments form a complex radial pattern without text/© Springer Health+ IME