CAJ | 학술논문

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.
수집북경협화의원대동맥염합병심기병변병례공13례,대기림상표현、실험실검사、초성심동도검사급치료、예후진행회고성분석.결과현시,환자적초성심동도주요표현위심장확대(좌심강확대위주)、미만성실벽운동감약화좌심실수축공능감저,기중2례피진단위확장형심기병.사용대제량당피질격소연합배린선알시주요치료방법.13례중1례환자사망,1례병정지속악화,여11례환자병정은정혹호전.대동맥염합병심기병변비교소견,발병년령편소、기병은닉、병정중、진전교쾌,림상응적겁평개심공능、완선초성심동도검사,급조발현화치료.조기대제량당피질격소연합배린선알치료유조우개선예후.
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.