背景在患有心脏结节病(CS)的人群中,大约三分之一缺乏心外受累,被认为患有孤立性CS。最近,日本循环学会更新了CS的诊断标准,提供一种诊断孤立CS的方法。我们旨在根据更新的日本循环学会指南,评估使用多模态成像方法诊断的孤立性CS的特征。方法和结果我们回顾性地确定了2012年至2019年间接受18F-氟代脱氧葡萄糖正电子发射断层扫描的161例疑似CS患者。根据指导方针,患者被分类为心外受累的CS,隔离CS,或者没有CS。我们比较了多模态成像的特征和主要不良心血管事件的发生率。日本循环学会标准将28例患者(17%)归类为CS,其中4例(2%)具有组织学确认,21(13%)作为分离的CS,112(70%)为无CS。与CS相比,孤立性CS显示左心室容积增加,左心室射血分数降低(均P<0.01)。在522天的中位随访期内,24例患者发生主要不良心血管事件。隔离CS(危险比,3.35;[95%CI,1.08-10.39],P=0.036)在调整左心室射血分数和类固醇降低后与主要不良心血管事件独立相关。在41例进行18F-氟代脱氧葡萄糖正电子发射断层扫描评估的患者亚组中,在18F-脱氧葡萄糖正电子发射断层扫描中,只有更新的CS标准与心肌炎症的改善相关.结论使用更新的日本循环协会指南检测到的孤立性CS与不良的无事件生存率相关,应谨慎管理。
Background In the population with cardiac sarcoidosis (CS), approximately one third lacks extracardiac involvement and is considered to have isolated CS. Recently, the Japanese Circulation Society updated the diagnostic criteria for CS, providing a methodology for diagnosing isolated CS. We aimed to assess the characteristics of isolated CS diagnosed using a multimodal imaging approach according to the updated Japanese Circulation Society
guidelines. Methods and Results We retrospectively identified 161 consecutive patients who underwent 18F-fluorodeoxyglucose positron emission tomography for suspected CS between 2012 and 2019. According to the
guidelines, patients were classified as having CS with extracardiac involvement, isolated CS, or no CS. We compared the characteristics of multimodality imaging and the prevalence of major adverse cardiovascular events. The Japanese Circulation Society criteria classified 28 patients (17%) as having CS with 4 (2%) with histological confirmation, 21 (13%) as isolated CS, and 112 (70%) as no CS. Compared with CS, isolated CS showed higher left ventricular volume and reduced left ventricular ejection fraction (P<0.01 for all). During the median follow-up period of 522 days, 24 patients had major adverse cardiovascular events. Isolated CS (hazard ratio, 3.35; [95% CI, 1.08-10.39], P=0.036) was independently associated with major adverse cardiovascular events after adjusting for reduced left ventricular ejection fraction and steroid. In the subgroup of 41 patients with serial 18F-fluorodeoxyglucose positron emission tomography evaluation, only updated CS criteria were associated with improvement in myocardial inflammation on 18F-fluorodeoxyglucose positron emission tomography. Conclusions Isolated CS detected using the updated Japanese Circulation Society
guidelines was associated with poor event-free survival and should be managed with caution.