Cardiac Magnetic Resonance Imaging Guided Risk Stratification in Cardiac Sarcoidosis

项目摘要/摘要 据估计，美国有20万人患有结节病。结节病是一种多系统肉芽肿性疾病。 病因不明。多达四分之一的结节病患者可能有心脏受累。心脏 结节病是与结节病相关的死亡的第二大原因，占多达四分之一 死于结节病。大多数心脏结节病的死亡是由于室性心律失常。 心血管磁共振成像经常用于评估疑似或 已知心脏结节病。在这项提案中，我们的目标是研究心血管磁共振成像 可能与可疑或已知心脏病患者不良结局相关的生物标志物 结节病。利用这些信息，我们将识别整合的表型--由来自 心血管磁共振成像、人口学和临床特征-与 心脏方面的不良后果。拟议的工作将建立心脏病的高风险和低风险表型。 预测从免疫抑制和/或植入型心脏复律除颤器获益的结节病患者 预防心律失常猝死和其他心脏结节病相关不良结局。这些 数据将为测试使用整合表型的策略的前瞻性研究奠定基础 选择心脏结节病患者进行治疗。有选择地和适当地使用这些治疗方法可以 改善心脏结节病患者的长期临床结果，特别是通过预防脑室结节病 与心律失常相关的心脏性猝死。

PROJECT SUMMARY/ABSTRACT An estimated 200,000 Americans live with sarcoidosis. Sarcoidosis is a multisystem granulomatous disease of unknown etiology. Up to a quarter of patients with sarcoidosis may have cardiac involvement. Cardiac sarcoidosis is the second leading cause of sarcoidosis-related mortality, accounting for up to a quarter of deaths from sarcoidosis. Most deaths from cardiac sarcoidosis are due to ventricular arrhythmias. Cardiovascular magnetic resonance imaging is frequently used in the evaluation of patients with suspected or known cardiac sarcoidosis. In this proposal, we aim to investigate cardiovascular magnetic resonance imaging biomarkers that may be associated with adverse outcomes in patients with suspected or known cardiac sarcoidosis. Using this information, we will identify integrative phenotypes – composed of biomarkers from cardiovascular magnetic resonance imaging, demographics, and clinical characteristics – associated with adverse cardiac outcomes. The proposed work will establish high- and low-risk phenotypes of cardiac sarcoidosis patients that predict benefit from immunosuppression and/or implantable cardioverter-defibrillators to prevent sudden arrhythmic cardiac death and other cardiac sarcoidosis-related adverse outcomes. These data will lay the groundwork for prospective studies testing the strategy of using the integrative phenotypes to select cardiac sarcoidosis patients for treatments. Selective and appropriate use of these treatments could improve long-term clinical outcomes in patients with cardiac sarcoidosis, in particular by preventing ventricular arrhythmia-related sudden cardiac death.