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Purpose of Review <p>Hot phases are a challenging clinical presentation in arrhythmogenic cardiomyopathy (ACM), marked by acute chest pain and elevated cardiac troponins in the absence of obstructive coronary disease. These episodes manifest as myocarditis and primarily affect young patients, contributing to a heightened risk of life-threatening arrhythmias and potential disease progression. This review aims to synthesize recent research on the pathophysiology, diagnostic challenges, and therapeutic management of hot phases in ACM.</p> Recent Findings <p>Hot phases have been linked to genetic mutations, particularly in desmosomal proteins such as Desmoplakin (<i>DSP</i>). Diagnostic challenges include differentiating hot phases from isolated acute myocarditis, through identification of red flags and a multimodal approach,&#xa0;including CMR, FDG-PET, endomyocardial biopsy and genetic testing. Emerging therapies, such as immunosuppressive and anti-inflammatory treatments, show promise in managing hot-phase episodes.</p> Summary <p>Hot phases in ACM present a significant risk for arrhythmias and disease progression, necessitating a comprehensive diagnostic and therapeutic management. A multimodal diagnostic&#xa0;approach&#xa0;is essential for accurate diagnosis, but further research is needed to refine these strategies and improve prognosis for affected patients.</p>

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

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年1月9日

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  • Giulia Bassetto,
  • Federico Angriman,
  • Carola Pio Loco detto Gava,
  • Alessia Paldino,
  • Maria Perotto,
  • Luca Bordignon,
  • Marta Gigli,
  • Matteo Dal Ferro,
  • Laura Massa,
  • Alessandro Altinier,
  • Antonio De Luca,
  • Gianfranco Sinagra,
  • Marco Merlo

摘要

Purpose of Review

Hot phases are a challenging clinical presentation in arrhythmogenic cardiomyopathy (ACM), marked by acute chest pain and elevated cardiac troponins in the absence of obstructive coronary disease. These episodes manifest as myocarditis and primarily affect young patients, contributing to a heightened risk of life-threatening arrhythmias and potential disease progression. This review aims to synthesize recent research on the pathophysiology, diagnostic challenges, and therapeutic management of hot phases in ACM.

Recent Findings

Hot phases have been linked to genetic mutations, particularly in desmosomal proteins such as Desmoplakin (DSP). Diagnostic challenges include differentiating hot phases from isolated acute myocarditis, through identification of red flags and a multimodal approach, including CMR, FDG-PET, endomyocardial biopsy and genetic testing. Emerging therapies, such as immunosuppressive and anti-inflammatory treatments, show promise in managing hot-phase episodes.

Summary

Hot phases in ACM present a significant risk for arrhythmias and disease progression, necessitating a comprehensive diagnostic and therapeutic management. A multimodal diagnostic approach is essential for accurate diagnosis, but further research is needed to refine these strategies and improve prognosis for affected patients.