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

IF 3.1 3区医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS

Current Cardiology Reports Pub Date : 2025-01-09 DOI:10.1007/s11886-024-02168-6

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

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Abstract

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. 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.

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热期心肌病：病理生理学、诊断挑战和新兴疗法。

回顾目的：热期是心律失常性心肌病（ACM）的一个具有挑战性的临床表现，在没有阻塞性冠状动脉疾病的情况下，以急性胸痛和心肌肌钙蛋白升高为特征。这些发作表现为心肌炎，主要影响年轻患者，增加了危及生命的心律失常和潜在疾病进展的风险。本文综述了ACM热期的病理生理学、诊断挑战和治疗管理方面的最新研究。最近的发现：热期与基因突变有关，特别是在桥粒蛋白中，如桥粒蛋白（DSP）。诊断挑战包括通过识别危险信号和多模式方法（包括CMR、FDG-PET、心内膜活检和基因检测），从孤立的急性心肌炎中区分热期。新兴疗法，如免疫抑制和抗炎治疗，在治疗热期发作方面显示出希望。ACM的热期存在心律失常和疾病进展的重大风险，需要全面的诊断和治疗管理。多模式诊断方法对于准确诊断至关重要，但需要进一步研究来完善这些策略并改善受影响患者的预后。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Current Cardiology Reports CARDIAC & CARDIOVASCULAR SYSTEMS-

CiteScore

6.20

自引率

2.70%

发文量

209

期刊介绍： The aim of this journal is to provide timely perspectives from experts on current advances in cardiovascular medicine. We also seek to provide reviews that highlight the most important recently published papers selected from the wealth of available cardiovascular literature. We accomplish this aim by appointing key authorities in major subject areas across the discipline. Section editors select topics to be reviewed by leading experts who emphasize recent developments and highlight important papers published over the past year. An Editorial Board of internationally diverse members suggests topics of special interest to their country/region and ensures that topics are current and include emerging research. We also provide commentaries from well-known figures in the field.