Müller Maximilian L, Poller Wolfgang, Skurk Carsten, Poddubnyy Denis, Siegmund Britta, Schneider Thomas, Landmesser Ulf, Heidecker Bettina
Department of Cardiology, Campus Benjamin Franklin, Charité-Universitätsmedizin Berlin corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Hindenburgdamm 30, 12203 Berlin, Germany.
Department of Gastroenterology, Infectious Diseases and Rheumatology, Campus Benjamin Franklin, Charité-Universitätsmedizin Berlin corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Hindenburgdamm 30, 12203 Berlin, Germany.
Eur Heart J Case Rep. 2023 Jan 11;7(1):ytad017. doi: 10.1093/ehjcr/ytad017. eCollection 2023 Jan.
Sarcoidosis is a granulomatous multi-organ disease of unknown aetiology. Despite being relatively rare, cardiac sarcoidosis constitutes a very important manifestation of sarcoidosis, as its symptoms regularly precede or occur in isolation of more prevalent ones, and as it is the main driver of mortality in systemic sarcoidosis.
We present the case of a 37-year-old woman, in which clinically isolated cardiac sarcoidosis revealed widespread systemic sarcoidosis. Apart from constitutional symptoms and strong recurrent dizziness (i.e. near-syncopes), which persisted for multiple years already, our patient initially presented with complex conduction abnormalities, including a right bundle branch block, left anterior hemi-block, and atrioventricular block °1. Following inconclusive endomyocardial biopsies, performed due to detection of focal septal scarring on cardiac magnetic resonance imaging, an F-FDG-PET-CT, performed upon admission to our clinic, showed distinct hypermetabolic lesions indicative of active inflammation in various organs and raised suspicion of systemic sarcoidosis. Eventually, histopathological evidence of non-caseating granulomas in affected lymph nodes, extracted by bronchoscopy, confirmed the diagnosis of systemic sarcoidosis after reasonable exclusion of other granulomatous diseases. Immediate initiation of long-term immunosuppressive therapy led to almost complete remission, as monitored by consequential F-FDG-PET-CT scans.
Unexplained complex conduction abnormalities in young patients may be a sign of sarcoidosis, even in isolation of more prevalent symptoms. Correct interpretation and prompt initiation of a structured interdisciplinary diagnostic workup, including F-FDG-PET-CT as the imaging modality of choice, are essential to initiate specific treatment and obviate the major risk of mortality resulting from cardiac sarcoidosis.
结节病是一种病因不明的肉芽肿性多器官疾病。尽管相对罕见，但心脏结节病是结节病的一种非常重要的表现形式，因为其症状通常先于或独立于更常见的症状出现，并且是系统性结节病死亡的主要驱动因素。
我们报告了一名37岁女性的病例，其中临床孤立的心脏结节病揭示了广泛的系统性结节病。除了持续多年的全身症状和强烈的反复头晕（即接近晕厥）外，我们的患者最初表现为复杂的传导异常，包括右束支传导阻滞、左前分支传导阻滞和一度房室传导阻滞。由于心脏磁共振成像检测到局灶性室间隔瘢痕，进行了心肌内膜活检，但结果不明确，入院时进行的F-FDG-PET-CT显示不同器官有明显的高代谢病变，提示有活动性炎症，怀疑为系统性结节病。最终，通过支气管镜检查提取的受累淋巴结非干酪样肉芽肿的组织病理学证据，在合理排除其他肉芽肿性疾病后，证实了系统性结节病的诊断。立即开始长期免疫抑制治疗，随后的F-FDG-PET-CT扫描监测显示几乎完全缓解。
年轻患者不明原因的复杂传导异常可能是结节病的征兆，即使没有更常见的症状。正确解读并迅速启动包括F-FDG-PET-CT作为首选成像方式的结构化多学科诊断检查，对于启动特异性治疗和避免心脏结节病导致的主要死亡风险至关重要。
