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End stage of arrhythmogenic cardiomyopathy with severe involvement of the interventricular septum

Maartje Noorman1, Judith A Groeneweg, Angeliki Asimaki

  • 1Department of Medical Physiology, University Medical Center Utrecht, Utrecht, The Netherlands.

Heart Rhythm
|October 23, 2012
PubMed

Insights

Arrhythmogenic cardiomyopathy, caused by a plakophilin-2 mutation, affects the entire heart, not just the right ventricle. This case study highlights extensive fibrofatty replacement and interventricular septum involvement in advanced disease.

Area of Science:

  • Cardiology
  • Genetics
  • Pathology

Background:

  • Arrhythmogenic cardiomyopathy (ACM) is a primary inherited heart muscle disease.
  • Typically, ACM is characterized by fibrofatty replacement of the right ventricle, leading to arrhythmias.
  • Genetic mutations, such as in plakophilin-2, are implicated in ACM pathogenesis.

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