Morales Juana Perez, Spaccavento Ana, Guerchicoff Marianna, Burgos Lucrecia, Costabel Juan Pablo, Cingolani Eugenio, de Lima Alberto Alves
Cardiomyopathies Clinic Cardiovascular Institute of Buenos Aires, Buenos Aires, Argentina.
Department of Cardiology, Cardiac Electrophysiology Section at Cedars-Sinai, Los Angeles, California, USA.
Am J Cardiol. 2025 May 1;242:37-40. doi: 10.1016/j.amjcard.2025.01.034. Epub 2025 Feb 8.
Genetic testing for cardiomyopathies has become a pivotal tool in diagnosing conditions with diverse and often overlapping clinical presentations. Arrhythmogenic cardiomyopathy (ACM), which affects approximately 1 in 5000 individuals, is a life-threatening condition associated with life-threatening arrhythmias and sudden cardiac death (SCD). While ACM typically presents with right- or left-sided heart failure, ventricular arrhythmias, or SCD, it can also manifest as acute or recurrent myocarditis. These inflammatory episodes, known as the "hot phase" of ACM, are less common but highly relevant in certain clinical contexts. We present the case of a 20-year-old male with recurrent episodes of acute myocarditis, confirmed by evidence of myocardial inflammation on cardiac magnetic resonance imaging (cMRI). Genetic testing revealed a pathogenic mutation in the desmoplakin (DSP) gene associated with ACM, raising the suspicion that the recurrent myocarditis episodes represent a "hot phase" of this inherited condition. In conclusion, this case underscores the importance of considering ACM in patients with recurrent myocarditis and highlights the role of genetic testing in uncovering underlying etiologies.
心肌病的基因检测已成为诊断临床表现多样且常常重叠病症的关键工具。致心律失常性心肌病(ACM)影响约五千分之一的人群,是一种与危及生命的心律失常和心源性猝死(SCD)相关的危及生命的病症。虽然ACM通常表现为右侧或左侧心力衰竭、室性心律失常或SCD,但它也可表现为急性或复发性心肌炎。这些炎症发作,即所谓的ACM“热期”,不太常见,但在某些临床情况下高度相关。我们报告一例20岁男性反复出现急性心肌炎发作的病例,心脏磁共振成像(cMRI)显示心肌炎症迹象,证实了这一情况。基因检测发现与ACM相关的桥粒斑蛋白(DSP)基因存在致病突变,这使人怀疑反复出现的心肌炎发作代表了这种遗传性疾病的“热期”。总之,该病例强调了在反复出现心肌炎的患者中考虑ACM的重要性,并突出了基因检测在揭示潜在病因方面的作用。