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12例巨细胞性心肌炎的临床识别与特征分析

Clinical Identification and Characteristic Analysis of Giant Cell Myocarditis in 12 Cases.

作者信息

Liu Shangyu, Zheng Lihui, Shen Lishui, Wu Lingmin, Yao Yan

机构信息

State Key Laboratory of Cardiovascular Disease, National Center for Cardiovascular Diseases, Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.

出版信息

Front Cardiovasc Med. 2021 Apr 13;8:649094. doi: 10.3389/fcvm.2021.649094. eCollection 2021.

Abstract

Giant cell myocarditis (GCM) is a rare, rapidly progressing cardiomyopathy with high mortality, if not diagnosed and treated in time. We analyzed the progression and clinical manifestations of patients with definitive diagnosis of GCM. We enrolled 12 patients diagnosed with GCM in the explanted heart during heart transplantation (HTx) or by endomyocardial biopsy (EMB) and collected information on demographic data, cardiac structure and function, arrhythmias, preliminary diagnosis, and delay of the diagnosis. Seven cases were diagnosed from biopsy samples during HTx, and five cases were diagnosed through EMB. Before the diagnosis of GCM based on pathological analysis, these patients had been incorrectly diagnosed with arrhythmogenic right ventricular cardiomyopathy ( = 5), dilated cardiomyopathy ( = 2), ventricular tachycardia ( = 2), viral myocarditis ( = 1), cardiac amyloidosis ( = 1), and ischemic cardiomyopathy ( = 1) based on clues such as symptoms, arrhythmia, and cardiac imaging. Patients diagnosed with GCM through EMB had a shorter symptom-onset-to-diagnosis time (6.6 ± 2.7 months) and milder heart damage (left ventricular ejection fraction, 47.2 ± 8.8%) than those diagnosed during HTx (11.0 ± 3.3 months, = 0.034; 31.4 ± 10.9%, = 0.024). GCM is easily misdiagnosed as other types of myocarditis and cardiomyopathy. Pathological examination of the myocardium is the most reliable diagnostic method for GCM. Endocardial biopsy can identify patients with GCM at an earlier stage.

摘要

巨细胞性心肌炎(GCM)是一种罕见的、进展迅速的心肌病,若不及时诊断和治疗,死亡率很高。我们分析了确诊为GCM的患者的病情进展和临床表现。我们纳入了12例在心脏移植(HTx)期间通过心脏移植或心内膜心肌活检(EMB)确诊为GCM的患者,并收集了人口统计学数据、心脏结构和功能、心律失常、初步诊断以及诊断延迟等信息。7例在HTx期间从活检样本中确诊,5例通过EMB确诊。在基于病理分析诊断为GCM之前,这些患者曾根据症状、心律失常和心脏成像等线索被误诊为致心律失常性右室心肌病(=5)、扩张型心肌病(=2)、室性心动过速(=2)、病毒性心肌炎(=1)、心脏淀粉样变性(=1)和缺血性心肌病(=1)。通过EMB诊断为GCM的患者从症状出现到诊断的时间较短(6.6±2.7个月),心脏损害较轻(左室射血分数,47.2±8.8%),而在HTx期间诊断的患者(11.0±3.3个月,P=0.034;31.4±10.9%,P=0.024)。GCM很容易被误诊为其他类型的心肌炎和心肌病。心肌病理检查是诊断GCM最可靠的方法。心内膜活检可以在早期识别GCM患者。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/94ea/8076517/93c52465c8c2/fcvm-08-649094-g0001.jpg

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