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大动脉炎合并心肌病变临床分析
引用本文:钟华,;朱卫国,;林雪,;费允云,;张文,;严晓伟,;张奉春.大动脉炎合并心肌病变临床分析[J].中华全科医师杂志,2014(11):929-932.
作者姓名:钟华  ;朱卫国  ;林雪  ;费允云  ;张文  ;严晓伟  ;张奉春
作者单位:[1]中国医学科学院北京协和医学院北京协和医院保健医疗部,100730; [2]中国医学科学院北京协和医学院北京协和医院普通内科,100730; [3]中国医学科学院北京协和医学院北京协和医院心内科,100730; [4]中国医学科学院北京协和医学院北京协和医院免疫内科,100730
摘    要:收集北京协和医院大动脉炎合并心肌病变病例共13例,对其临床表现、实验室检查、超声心动图检查及治疗、预后进行回顾性分析.结果显示,患者的超声心动图主要表现为心脏扩大(左心腔扩大为主)、弥漫性室壁运动减弱和左心室收缩功能减低,其中2例被诊断为扩张型心肌病.使用大剂量糖皮质激素联合环磷酰胺是主要治疗方法.13例中1例患者死亡,1例病情持续恶化,余11例患者病情稳定或好转.大动脉炎合并心肌病变比较少见,发病年龄偏小、起病隐匿、病情重、进展较快,临床应积极评价心功能、完善超声心动图检查,及早发现和治疗.早期大剂量糖皮质激素联合环磷酰胺治疗有助于改善预后.

关 键 词:Takayasu动脉炎  心肌疾病

Clinical analysis of Takayasu's arteritis with cardiomyopathy
Institution:Zhong Hua, Zhu Weiguo, Lin Xue, Fei Yunyun, Zhang Wei, Yan Xiaowei, Zhang Fengchun( Department of Health Care, Peking Union Hospital, Chinese Academy of Medical Sciences, & Peking Union Medical College, Beijing 100730, China)
Abstract:The clinical data of 13 patients diagnosed as Takayasu's arteritis with cardiomyopathy in Peking Union Hospital were reviewed.Echocardiography showed cardiomegaly (left chambers mainly),diffuse weakened myocardial wall movement and reduced left ventricular ejection fraction.Dilated cardiomyopathy was diagnosed in two patients.Glucocorticoid and cyclophosphamide were the essential medication for treatment.One patient died,1 patient was aggravated and the disease condition in remaining 11 patients was stable or improved.Takayasu's arteritis with cardiomyopathy is a rare disease and it attacks younger people.It is difficult to identify at onset,and the disease deteriorates rapidly.Echocardiography should be performed earlier to evaluate the heart function for early diagnosis and treatment.Administration of large dose of glucocorticoid and cyclophosphamide at early stage may improve the prognosis.
Keywords:Takayasu's arteritis  Cardiomyopathies
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