Spillmann Frank, Kühl Uwe, Van Linthout Sophie, Dominguez Fernando, Escher Felicitas, Schultheiss Heinz-Peter, Pieske Burkert, Tschöpe Carsten
Department of Cardiology Charité-University Medicine of Berlin, Campus Virchow Klinikum Berlin Germany.
Berlin-Brandenburg Center for Regenerative Therapies (BCRT)Charité-University Medicine of Berlin, Campus Virchow KlinikumBerlinGermany; Deutsches Zentrum für Herz-Kreislaufforschung (DZHK)-Standort Berlin/CharitéBerlinGermany.
ESC Heart Fail. 2016 Jun;3(2):138-142. doi: 10.1002/ehf2.12072. Epub 2015 Dec 9.
We report the case of a 17-year-old female patient with known hypertrophic cardiomyopathy and a Wolff-Parkinson-White syndrome. She came to our department for further evaluation of a new diagnosed dilated cardiomyopathy characterized by an enlargement of the left ventricle and a fall in ejection fraction. Clinically, she complained about atypical chest pain, arrhythmic episodes with presyncopal events, and dyspnea (NYHA III) during the last 6 months. Non-invasive and invasive examinations including magnetic resonance imaging, electrophysiological examinations, and angiography did not lead to a conclusive diagnosis. Therefore, endomyocardial biopsies (EMBs) were taken to investigate whether a specific myocardial disease caused the impairment of the left ventricular function. EMB analysis resulted in the diagnosis of a virus-negative, active myocarditis. Based on this diagnosis, an immunosuppressive treatment with prednisolone and azathioprine was started, which led to an improvement of cardiac function and symptoms within 3 months after initiating therapy. In conclusion, we show that external stress triggered by myocarditis can induce a reversible transition from a hypertrophic cardiomyopathy to a dilated cardiomyopathy phenotype. This case strongly underlines the need for a thorough and invasive examination of heart failure of unknown causes, including EMB investigations as recommend by the actual ESC position statement.
我们报告了一例17岁女性患者,她患有肥厚型心肌病和预激综合征。她因新诊断的扩张型心肌病前来我院进一步评估,其特征为左心室扩大和射血分数下降。临床上,她在过去6个月中出现非典型胸痛、伴有晕厥前期事件的心律失常发作以及呼吸困难(纽约心脏协会III级)。包括磁共振成像、电生理检查和血管造影在内的非侵入性和侵入性检查均未得出明确诊断。因此,进行了心内膜心肌活检(EMB),以调查是否存在特定的心肌疾病导致左心室功能受损。EMB分析诊断为病毒阴性的活动性心肌炎。基于这一诊断,开始使用泼尼松龙和硫唑嘌呤进行免疫抑制治疗,治疗开始后3个月内心脏功能和症状得到改善。总之,我们表明心肌炎引发的外部应激可诱导从肥厚型心肌病到扩张型心肌病表型的可逆转变。该病例强烈强调了对病因不明的心力衰竭进行全面和侵入性检查的必要性,包括按照欧洲心脏病学会(ESC)现行立场声明的建议进行EMB检查。