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Hot Phases Cardiomyopathy: Pathophysiology, Diagnostic Challenges, and Emerging Therapies

Giulia Bassetto1, Federico Angriman2, Carola Pio Loco Detto Gava2

  • 1Center for Diagnosis and Treatment of Cardiomyopathies, Cardiovascular Department, Azienda Sanitaria Universitaria Giuliano Isontina (ASUGI), European Reference Network for Rare, University of Trieste, Via P. Valdoni 7, 34100, Trieste, Italy. bassettogiulia7@gmail.com.

Current Cardiology Reports
|January 9, 2025
PubMed

Insights

Hot phases in arrhythmogenic cardiomyopathy (ACM) involve myocarditis-like symptoms in young patients, increasing arrhythmia risk. Early diagnosis using genetic testing and advanced imaging, alongside new therapies, is crucial for better outcomes.

Area of Science:

  • Cardiology
  • Genetics
  • Pathophysiology

Background:

  • Hot phases in arrhythmogenic cardiomyopathy (ACM) present as acute chest pain and elevated troponins, mimicking myocarditis.
  • These episodes are linked to genetic mutations and pose a significant risk for arrhythmias and disease progression, especially in young patients.

Purpose of the Study:

  • To review current research on the pathophysiology of ACM hot phases.
  • To explore diagnostic challenges and emerging therapeutic strategies for ACM hot phases.

Main Methods:

  • Literature review synthesizing recent findings on ACM hot phases.
  • Discussion of diagnostic tools including cardiac magnetic resonance (CMR), FDG-PET, endomyocardial biopsy, and genetic testing.
  • Analysis of emerging treatments such as immunosuppressive and anti-inflammatory therapies.

Main Results:

  • Hot phases are associated with genetic mutations, notably in desmosomal proteins like Desmoplakin (DSP).
  • Differentiating ACM hot phases from isolated myocarditis requires identifying red flags and employing a multimodal diagnostic approach.
  • Emerging therapies show promise, but further research is needed to refine diagnostic and therapeutic strategies.

Conclusions:

  • Hot phases in ACM represent a critical clinical challenge requiring comprehensive management.
  • A multimodal diagnostic approach is essential for accurate diagnosis and risk stratification.
  • Continued research is vital to improve prognosis and treatment efficacy for patients experiencing ACM hot phases.
Abstract

Keywords:
Arrhythmic riskCardiomyopathyDesmoplakinHot-phasesInflammationMyocarditis

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