Matsuda Takahide, Kuyama Naoto, Tsuruta Yuichiro, Takashio Seiji, Oda Seitaro, Hanatani Shinsuke, Kanazawa Hisanori, Yamamoto Eiichiro, Izumiya Yasuhiro, Tsujita Kenichi
Department of Cardiovascular Medicine, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan.
Department of Cardiovascular Medicine, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan.
JACC Case Rep. 2025 Sep 9:105354. doi: 10.1016/j.jaccas.2025.105354.
Cardiac sarcoidosis (CS) usually affects the left ventricle and presents with nonspecific features like conduction abnormalities and ventricular arrhythmias. However, right ventricle (RV)-dominant involvement has been increasingly reported, making diagnosis difficult.
A 55-year-old man presented with palpitations. Electrocardiography showed bifascicular block, first-degree atrioventricular block, and epsilon waves. Echocardiography revealed RV dilatation and dysfunction, suggesting arrhythmogenic right ventricular cardiomyopathy. However, computed tomography and fluorodeoxyglucose positron emission tomography revealed mediastinal and hilar lymphadenopathy with cardiac uptake. Myocardial biopsy confirmed epithelioid granulomas, diagnosing sarcoidosis. Prednisolone resolved cardiac inflammation, but ventricular tachycardia progressed, requiring cardioverter-defibrillator implantation.
RV-dominant CS can mimic arrhythmogenic right ventricular cardiomyopathy due to overlapping features. This case demonstrates the importance of multimodal imaging and biopsy for accurate diagnosis and highlights the need for arrhythmia monitoring even after inflammation subsides.
TAKE-HOME MESSAGE: Multimodal assessment is key to differentiating RV-dominant CS from arrhythmogenic right ventricular cardiomyopathy, and early diagnosis with appropriate management is essential for better outcomes.
心脏结节病（CS）通常累及左心室，表现为传导异常和室性心律失常等非特异性特征。然而，右心室（RV）为主受累的情况报道日益增多，这使得诊断变得困难。
一名55岁男性因心悸就诊。心电图显示双分支阻滞、一度房室传导阻滞及ε波。超声心动图显示右心室扩张和功能障碍，提示致心律失常性右心室心肌病。然而，计算机断层扫描和氟脱氧葡萄糖正电子发射断层扫描显示纵隔和肺门淋巴结肿大并伴有心脏摄取。心肌活检证实为上皮样肉芽肿，诊断为结节病。泼尼松龙减轻了心脏炎症，但室性心动过速进展，需要植入心脏复律除颤器。
右心室为主的心脏结节病由于特征重叠可酷似致心律失常性右心室心肌病。本病例表明多模态成像和活检对准确诊断的重要性，并强调即使炎症消退后仍需进行心律失常监测。
多模态评估是区分右心室为主的心脏结节病和致心律失常性右心室心肌病的关键，早期诊断并进行适当治疗对改善预后至关重要。
