Department of Cardiology Sahlgrenska University Hospital Gothenburg Sweden.
Institute of Medicine at Sahlgrenska Academy University of Gothenburg Gothenburg Sweden.
J Am Heart Assoc. 2023 Aug;12(15):e029481. doi: 10.1161/JAHA.123.029481. Epub 2023 Jul 25.
Background Cardiac involvement can be an initial manifestation in sarcoidosis. However, little is known about the association between various clinical phenotypes of cardiac sarcoidosis (CS) and outcomes. We aimed to analyze the relation of different clinical manifestations with outcomes of CS and to investigate the relative importance of clinical features influencing overall survival. Methods and Results A retrospective cohort of 141 patients with CS enrolled at 2 Swedish university hospitals was studied. Presentation, imaging studies, and outcomes of de novo CS and previously known extracardiac sarcoidosis were compared. Survival free of primary composite outcome (ventricular arrhythmias, heart transplantation, or death) was assessed. Machine learning algorithm was used to study the relative importance of clinical features in predicting outcome. Sixty-two patients with de novo CS and 79 with previously known extracardiac sarcoidosis were included. De novo CS showed more advanced New York Heart Association class (=0.02), higher circulating levels of NT-proBNP (N-terminal pro-B-type natriuretic peptide) (<0.001), and troponins (<0.001), as well as a higher prevalence of right ventricular dysfunction (<0.001). During a median (interquartile range) follow-up of 61 (44-77) months, event-free survival was shorter in patients with de novo CS (<0.001). The top 5 features predicting worse event-free survival in order of importance were as follows: impaired tricuspid annular plane systolic excursion, de novo CS, reduced right ventricular ejection fraction, absence of β-blockers, and lower left ventricular ejection fraction. Conclusions Patients with de novo CS displayed more severe disease and worse outcomes compared with patients with previously known extracardiac sarcoidosis. Using machine learning, right ventricular dysfunction and de novo CS stand out as strong overall predictors of impaired survival.
心脏受累可作为肉样瘤病的首发表现。然而，人们对不同临床表型的心肌肉样瘤病（CS）与结局之间的关联知之甚少。我们旨在分析不同临床表现与 CS 结局的关系，并探讨影响总生存的临床特征的相对重要性。
对在瑞典 2 所大学医院就诊的 141 例 CS 患者进行了回顾性队列研究。比较了新发 CS 和先前已知的心脏外肉样瘤病的表现、影像学研究和结局。评估了无主要复合结局（室性心律失常、心脏移植或死亡）的生存情况。使用机器学习算法研究了临床特征在预测结局方面的相对重要性。纳入 62 例新发 CS 和 79 例先前已知的心脏外肉样瘤病患者。新发 CS 患者纽约心脏协会（NYHA）心功能分级更高（=0.02），循环 N 末端 B 型利钠肽（NT-proBNP）水平更高（<0.001），肌钙蛋白水平更高（<0.001），右心室功能障碍更常见（<0.001）。中位（四分位距）随访 61（44-77）个月期间，新发 CS 患者的无事件生存时间更短（<0.001）。按重要性顺序，预测无事件生存时间更差的前 5 个特征如下：三尖瓣环平面收缩期位移受损、新发 CS、右心室射血分数降低、无β受体阻滞剂、左心室射血分数较低。
与先前已知的心脏外肉样瘤病患者相比，新发 CS 患者的疾病更严重，结局更差。使用机器学习，右心室功能障碍和新发 CS 是总体生存预后不良的强有力预测指标。
