Tan Jian Liang, Sharma Sandeep K
Department of Internal Medicine, Crozer-Chester Medical Center, 1 Medical Center Blvd, Upland, PA, USA.
Department of Cardiology, Crozer-Chester Medical Center, Upland, PA, USA.
Eur Heart J Case Rep. 2018 Oct 11;2(4):yty103. doi: 10.1093/ehjcr/yty103. eCollection 2018 Dec.
Cardiac sarcoidosis (CS) accounts for a substantial morbidity and mortality. Early recognition of CS is important to prevent such detrimental consequences. A definite diagnosis of cardiac sarcoidosis remains challenging. Even after the diagnosis of CS is established, the appropriate dose and duration of corticosteroids in the treatment of CS have not been well-defined.
In this report, we discuss a case of a 50-year-old man who presented with recurrent syncope. Electrocardiogram revealed sinus rhythm with left bundle branch block. Telemetry captured high-grade atrioventricular block. Coronary angiogram showed no coronary artery disease. Left ventriculography revealed left ventricular ejection fraction (LVEF) of 35-40%. A dual-chamber pacemaker was implanted. Cardiac magnetic resonance revealed mid-myocardial scarring suggestive of sarcoidosis. Computed tomography of the chest showed lymphadenopathy. Transbronchial biopsy was unrevealing; however, mediastinoscopy and lymph node biopsy showed non-caseating granulomas diagnostic of sarcoidosis. He became pacemaker dependent as noted in outpatient pacemaker interrogations. A biventricular implantable cardioverter-defibrillator upgrade was performed for primary prevention of sudden cardiac death. He was started on prednisone taper over the course of 6 months. After 1-year, his LVEF improved to 55% and native atrioventricular (AV) conduction had recovered as noted in outpatient device interrogations.
This case highlights the importance to include CS in the differential diagnosis of a young patient with conduction system disease and non-ischaemic cardiomyopathy for appropriate treatment. Patients with left ventricular systolic dysfunction and AV nodal disease could potentially benefit from a slow prednisone taper over the course of 6 months.
心脏结节病（CS）会导致较高的发病率和死亡率。早期识别CS对于预防此类不良后果很重要。心脏结节病的明确诊断仍然具有挑战性。即使在确诊CS后，治疗CS时皮质类固醇的合适剂量和疗程也尚未明确。
在本报告中，我们讨论了一名50岁男性反复晕厥的病例。心电图显示窦性心律伴左束支传导阻滞。遥测记录到高度房室传导阻滞。冠状动脉造影显示无冠状动脉疾病。左心室造影显示左心室射血分数（LVEF）为35%-40%。植入了双腔起搏器。心脏磁共振显示心肌中层瘢痕形成，提示结节病。胸部计算机断层扫描显示淋巴结病。经支气管活检未发现异常；然而，纵隔镜检查和淋巴结活检显示非干酪样肉芽肿，可诊断为结节病。门诊起搏器程控显示他变得依赖起搏器。为预防心脏性猝死进行了双心室植入式心脏复律除颤器升级。在6个月的时间里逐渐减少泼尼松剂量。1年后，门诊设备程控显示他的LVEF提高到55%，并且恢复了自身房室（AV）传导。
该病例强调了在年轻的传导系统疾病和非缺血性心肌病患者的鉴别诊断中纳入CS以进行适当治疗的重要性。左心室收缩功能障碍和房室结疾病患者可能会从6个月内逐渐减少泼尼松剂量中获益。
