Division of Cardiology, University of Ottawa Heart Institute, 40 Ruskin Street, Ottawa, ON, Canada K1Y 4 W7.
Division of Cardiology, Heart and Lung Center, Helsinki University Hospital, Helsinki, Finland.
Eur Heart J. 2017 Sep 14;38(35):2663-2670. doi: 10.1093/eurheartj/ehw328.
Approximately 5% of patients with sarcoidosis will have clinically manifest cardiac involvement presenting with one or more of ventricular arrhythmias, conduction abnormalities, and heart failure. Cardiac presentations can be the first (and/or an unrecognized) manifestation of sarcoidosis in a variety of circumstances. Cardiac symptoms are usually dominant over extra-cardiac as most patients with clinically manifest disease have minimal extra-cardiac disease and up to two-thirds have isolated cardiac sarcoidosis (CS). It is estimated that another 20-25% of pulmonary/systemic sarcoidosis patients have asymptomatic cardiac involvement (clinically silent disease). The extent of left ventricular dysfunction seems to be the most important predictor of prognosis among patients with clinically manifest CS. In addition, the extent of myocardial late gadolinium enhancement is emerging as an important prognostic factor. The literature shows some controversy regarding outcomes for patients with clinically silent CS and larger studies are needed. Immunosuppression therapy (usually with corticosteroids) has been suggested for the treatment of clinically manifest CS despite minimal data supporting it. Fluorodeoxyglucose Positron Emission Tomography imaging is often used to detect active disease and guide immunosuppression. Patients with clinically manifest disease often need device therapy, typically with implantable cardioverter defibrillators.
大约 5%的结节病患者会出现临床明显的心脏受累,表现为室性心律失常、传导异常和心力衰竭。心脏表现可以是各种情况下结节病的首发(和/或未被识别)表现。心脏症状通常比心脏外表现更为突出,因为大多数有临床明显疾病的患者仅有轻微的心脏外疾病,多达三分之二的患者有孤立性心脏结节病(CS)。据估计,另外 20-25%的肺/全身结节病患者有无症状性心脏受累(临床无症状疾病)。左心室功能障碍的程度似乎是临床明显 CS 患者预后的最重要预测因素。此外,心肌晚期钆增强的程度正在成为一个重要的预后因素。文献显示,对于临床无症状 CS 患者的结局存在一些争议,需要更大规模的研究。尽管支持它的数据很少,但免疫抑制治疗(通常使用皮质类固醇)已被建议用于治疗临床明显的 CS。氟脱氧葡萄糖正电子发射断层扫描成像常用于检测活动性疾病并指导免疫抑制治疗。有临床明显疾病的患者通常需要器械治疗,通常是植入式心脏复律除颤器。