The BioMedical Engineering and Imaging Institute (BMEII), Icahn School of Medicine at Mount Sinai, New York, New York; Cardiovascular Institute, Icahn School of Medicine at Mount Sinai, New York, New York.
Section of Respiratory Diseases, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padova, Padua, Italy.
J Am Coll Cardiol. 2020 Oct 20;76(16):1878-1901. doi: 10.1016/j.jacc.2020.08.042.
Sarcoidosis is a complex disease with heterogeneous clinical presentations that can affect virtually any organ. Although the lung is typically the most common organ involved, combined pulmonary and cardiac sarcoidosis (CS) account for most of the morbidity and mortality associated with this disease. Pulmonary sarcoidosis can be asymptomatic or result in impairment in quality of life and end-stage, severe, and/or life-threatening disease. The latter outcome is seen almost exclusively in those with fibrotic pulmonary sarcoidosis, which accounts for 10% to 20% of pulmonary sarcoidosis patients. CS is problematic to diagnose and may cause significant morbidity and death from heart failure or ventricular arrhythmias. The diagnosis of CS usually requires surrogate cardiac imaging biomarkers, as endomyocardial biopsy has relatively low yield, even with directed electrophysiological mapping. Treatment of CS is often multifactorial, involving a combination of antigranulomatous therapy and pharmacotherapy for cardiac arrhythmias and/or heart failure in addition to device placement and cardiac transplantation.
结节病是一种复杂的疾病,临床表现具有异质性,几乎可影响任何器官。虽然肺部通常是最常受累的器官,但肺和心脏结节病(CS)合并症占与该疾病相关的大多数发病率和死亡率。肺部结节病可能无症状,也可能导致生活质量受损以及终末期、严重和/或危及生命的疾病。后一种结果几乎仅见于纤维化性肺部结节病患者,占肺部结节病患者的 10%至 20%。CS 的诊断具有挑战性,可能导致心力衰竭或室性心律失常引起的显著发病率和死亡。CS 的诊断通常需要替代心脏成像生物标志物,因为即使进行了定向电生理学图谱,心内膜心肌活检的产量也相对较低。CS 的治疗通常是多因素的,除了器械放置和心脏移植外,还包括抗肉芽肿治疗和针对心律失常和/或心力衰竭的药物治疗。