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在左心室辅助装置植入或心脏移植时诊断为心肌结节病患者的自然病史。

Natural History of Patients Diagnosed with Cardiac Sarcoidosis at Left Ventricular Assist Device Implantation or Cardiac Transplantation.

机构信息

From the Department of Internal Medicine.

Department of Cardiovascular Medicine.

出版信息

ASAIO J. 2021 May 1;67(5):583-587. doi: 10.1097/MAT.0000000000001262.

DOI:10.1097/MAT.0000000000001262
PMID:33902104
Abstract

To our knowledge, natural history has not been reported for cardiac sarcoidosis (CS) diagnosed by pathologic evaluation of the apical core at left ventricular assist device (LVAD) implantation or cardiac transplantation. We retrospectively identified 232 consecutive patients meeting CS criteria. Of these patients, 54 were diagnosed by pathologic confirmation of CS, 10 after evaluation of the apical core (LVAD implant) or explanted heart (transplant). We compared clinical characteristics at initial evaluation and outcomes for these 10 patients with those of 10 patients with known CS before LVAD implant/transplant. In the study group, five patients (50%) had confirmed extracardiac sarcoidosis before LVAD implant/transplant; five had not been diagnosed with sarcoidosis. Mean (standard deviation) left ventricular ejection fraction at initial evaluation was 23% (16%), and left ventricular end-diastolic dimension was 61 (10) mm. Four patients died during follow-up; however, no survival difference was found for the 10 patients diagnosed incidentally and the group with a previous diagnosis or institutional LVAD/transplant cohorts. Patients diagnosed with CS on pathological examination of the apical core/explanted heart may have severe dilated cardiomyopathy as the initial presentation. Outcomes for patients with CS after advanced heart failure therapies may be comparable with those of non-CS patients.

摘要

据我们所知,在左心室辅助装置 (LVAD) 植入或心脏移植时通过对心尖芯部进行病理评估诊断的心脏结节病 (CS) 尚未报道其自然病史。我们回顾性地确定了符合 CS 标准的 232 例连续患者。其中,54 例通过 CS 的病理证实诊断,10 例通过对心尖芯部(LVAD 植入)或移植心脏进行评估诊断。我们比较了这 10 例患者的初始评估临床特征和结局,以及在 LVAD 植入/移植前已知 CS 的 10 例患者的特征和结局。在研究组中,5 例患者(50%)在 LVAD 植入/移植前已确诊为心脏外结节病;5 例患者未被诊断为结节病。初始评估时左心室射血分数的平均值(标准差)为 23%(16%),左心室舒张末期内径为 61(10)mm。4 例患者在随访期间死亡;然而,在意外诊断的 10 例患者和有既往诊断或机构性 LVAD/移植队列的患者中,未发现生存差异。在心尖芯部/移植心脏的病理检查中诊断为 CS 的患者可能以严重扩张型心肌病为首发表现。在晚期心力衰竭治疗后 CS 患者的结局可能与非 CS 患者的结局相当。

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