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Desmoplakin Cardiomyopathy

Daniel D Han1, Gregory Jew2, Katherine Kaproth-Joslin3,4

  • 1Medical Scientist Training Program, University of Rochester School of Medicine and Dentistry, Rochester, NY, USA.

The International Journal of Cardiovascular Imaging
|March 9, 2026
PubMed

Insights

Desmoplakin (DSP) cardiomyopathy, a genetic heart condition from DSP gene mutations, causes severe heart problems. Cardiac MRI findings may mimic other heart diseases, requiring careful diagnosis.

Area of Science:

  • Cardiology
  • Genetics
  • Medical Imaging

Background:

  • Desmoplakin (DSP) cardiomyopathy is an inherited cardiac condition.
  • It stems from mutations in the desmosome gene DSP.
  • Consequences include arrhythmias, heart failure, and sudden cardiac death.

Purpose of the Study:

  • To highlight the diagnostic challenges of Desmoplakin (DSP) cardiomyopathy.
  • To differentiate its characteristic Cardiac MRI findings from other myocardial diseases.

Main Methods:

  • Review of clinical presentations of Desmoplakin (DSP) cardiomyopathy.
  • Analysis of Cardiac MRI features, specifically late gadolinium enhancement patterns.

Main Results:

  • Desmoplakin (DSP) cardiomyopathy presents with arrhythmias and heart failure.
  • Cardiac MRI typically shows a distinct ring-like subepicardial late gadolinium enhancement.
  • This pattern can be misdiagnosed as myocarditis or fatty infiltration.

Conclusions:

  • Accurate diagnosis of Desmoplakin (DSP) cardiomyopathy is crucial.
  • Recognizing the specific Cardiac MRI "ring" enhancement pattern aids differentiation.
  • Distinguishing DSP cardiomyopathy from mimics like myocarditis is vital for appropriate patient management.
Keywords:
Desmoplakin Cardiomyopahty

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