Work

Emotional stress-induced hot phases in a patient with a desmoplakin-associated arrhythmogenic cardiomyopathy: a case report

Year: 2026

Type: article

Abstract: Background: Desmoplakin (DSP) mutations are a common cause of arrhythmogenic cardiomyopathy (ACM), frequently presenting with recurrent myocarditis-like 'hot phases' that contribute to myocardial injury and increased arrhythmic risk. Case Summary: A 40-year-old woman presented with acute chest pain and marked troponin elevation following emotional stress. Her history was notable for recurrent stress-related myocarditis episodes and a diagnosis of biventricular arrhythmogenic cardiomyopathy due to a pathogenic DSP mutation. Cardiac magnetic resonance demonstrated diffuse myocardial oedema and a characteristic circumferential subepicardial ring-like pattern of late gadolinium enhancement, consistent with an active hot phase. Given the high-risk pathogenic DSP mutation with multiple high-risk features, including documented non-sustained ventricular tachycardia, frequent ventricular ectopy, and mild left ventricular disfunction (resulting in a calculated 5-year arrhythmic risk of 12% according to the novel DSP risk score)-and the presence of frequent hot phases recurrences and high burden of late gadolinium enhancement-a primary prevention implantable cardioverter-defibrillator was implanted after clinical stabilization. Medical therapy was optimized with β-blocker up-titration and low-dose selective serotonin reuptake inhibitor therapy targeting recurrent stress-related triggers. At follow-up, the patient reported sustained clinical well-being with no recurrence of hot phases or arrhythmic events. Conclusion: This case exemplifies DSP-related arrhythmogenic cardiomyopathy marked by recurrent, stress-triggered 'hot phases,' highlighting the pivotal interplay between inflammation and arrhythmic risk, and underscoring the need for refined, disease-specific risk stratification and targeted management strategies.

Source: European Heart Journal - Case Reports

DOI: 10.1093/ehjcr/ytag588

Authors: Annalisa Caputo, Lucia Ilaria Birtolo, Elena Sofia Canuti, Gianmarco Scoccia, Carmine Dario Vizza

Institutions: Policlinico Umberto I, Sapienza University of Rome

Language: English

Topic: Cardiovascular Effects of Exercise

Subfield: Cardiology and Cardiovascular Medicine

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: gold

Estimated APC paid: $815