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14:39

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Inflammatory Hot Phases in Arrhythmogenic Cardiomyopathy: A Dynamic Electrophysiologic Substrate Beyond Scar

Mehrdad Zarghami1, Neda Dianati Maleki2, Sajieh Naddaf3

  • 1Department of Internal Medicine, Jamaica Hospital Medical Center, Queens, New York, USA; Thrombosis Research Group, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.

JACC. Advances
|August 21, 2026
PubMed

Insights

Arrhythmogenic cardiomyopathy may involve "hot phases" with inflammation mimicking myocarditis, accelerating injury. Current management focuses on standard care, with immunomodulatory therapy still under investigation.

Area of Science:

  • Cardiology
  • Genetics
  • Immunology

Background:

  • Arrhythmogenic cardiomyopathy (ACM) is traditionally viewed as a progressive inherited heart condition.
  • Recent evidence suggests episodic inflammatory
  • hot phases
  • potentially mimicking acute myocarditis and worsening ACM progression.

Purpose of the Study:

  • To review evidence supporting the ACM hot-phase paradigm.
  • To propose a structured approach for evaluating suspected ACM hot phases.
  • To discuss current and future therapeutic strategies.

Main Methods:

  • Synthesis of mechanistic, imaging, genetic, and electrophysiologic data.
  • Review of cardiac magnetic resonance (CMR) tissue characterization techniques (T2 mapping, LGE).
  • Analysis of proposed pathomechanisms including inflammasome activation and immune responses.

Main Results:

  • The ACM hot phase is characterized by active inflammation and edema, distinct from chronic fibrofatty scar.
  • CMR imaging can differentiate active inflammation from chronic changes.
  • Proposed mechanisms involve genetic predisposition, immune system activation, and cellular stress pathways.

Conclusions:

  • The ACM hot-phase concept offers a new framework for understanding disease progression.
  • Suspected hot phases warrant comprehensive evaluation including genetic testing and family screening.
  • Standard ACM care is recommended, while immunomodulatory therapies require further research.

Keywords:
NLRP3 inflammasomearrhythmogenic cardiomyopathycardiac MRIhot phaseventricular tachycardia

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