心肌淀粉样变性的临床表现及MRI特征  

Clinical features and MRI characteristics in patients with cardiac amyloidosis

作　　者：万俊义[1] 赵世华[1] 蒋世良[1] 张岩[1] 闫朝武[1] 陆敏杰[1] 周阳[1] 黄洁[2] 赵红[3] 

机构地区：[1]中国医学科学院北京协和医学院阜外心血管病医院心血管病研究所放射科,100037 [2]中国医学科学院北京协和医学院阜外心血管病医院心血管病研究所内科,100037 [3]中国医学科学院北京协和医学院阜外心血管病医院心血管病研究所病理科,100037

出　　处：《中华放射学杂志》2010年第12期1297-1299,共3页Chinese Journal of Radiology

基　　金：国家自然科学基金资助项目（81071199）;首都医学发展科研基金联合公关项目（2009-1004）

摘　　要：目的 探讨心肌淀粉样变性临床表现及MRI特征.方法 回顾性分析5例经组织病理检查确诊的心肌淀粉样变性患者,其中3例经心脏移植,2例经心内膜活检证实.5例患者均经心电图、X线胸片、超声心动图和MR检查.结果 5例均确诊为心肌淀粉样变性,男女比例为4∶1,5例患者均有心电图异常;X线胸片3例有肺淤血,5例心脏增大,3例伴有少至中、大量胸腔积液;超声心动图示心房扩大,左心室壁肥厚,心室舒张受限,部分出现心肌回声增强;MRI表现为左心室壁普遍增厚,但以室间隔增厚为主,左心室射血分数32.5%±15.0%,限制性心室舒张期充盈受限;延迟增强MRI 4例表现为全心心内膜下为主的弥漫性粉尘样强化,同时累及乳头肌,其中3例患者室间隔左、右心室壁心内膜下均出现强化,在室间隔形成＂斑马征＂,1例表现左心室透壁性强化,自内向外强化程度渐弱.结论 MRI＂一站式＂扫描能够全面显示心肌淀粉样变性心脏结构和功能变化,心肌弥漫性粉尘样强化和＂斑马征＂,在心肌淀粉样变性诊断中具有很高的参考价值.Objective To observe the clinical features and cardiac magnetic resonance （CMR）imaging characteristics in patients with cardiac amyloidosis. Methods A total of 5 patients （4 males and 1 female） with the diagnosis of cardiac amyloidosis （3 were proven by heart transplantation, 2 by endomyocardial biopsy） were evaluated by electrocardiogram, echocardiogram, chest X-ray and CMR with delayed Gadolinium enhancement. Results Echocardiograms were abnormal in all five patients; chest X-ray showed pulmonary hemorrhage （ 3 ）, cardiomegaly （5）, pleural effusion （3）; echocardiogram showed atrial enlargement, left ventricular wall thickening, limited ventricular wall motion, etc. CMR exhibited increased thickness of the left ventricular wall, mild to moderate depression of systolic function （ mean ejection fraction： 32.5% ± 15.0% ） and bilateral atrial enlargement with restriction of diastolic ventricular filling. In all patients, there were widespread enhancement of the thickened myocardium on delayed postcontrast studies. In 4 patients, global subendocardial delayed gadolinium enhancement was found, in papillary muscles, and interventricular septa with＂ zebra-like＂ sign in 3 patients. Left ventricular transmural delayed gadolinium enhancement was found in 1 patient. Conclusions CMR shows a characteristic pattern of global subendocardial delayed gadolinium enhancement in cardiac amyloidosis. The findings may be valuable in the diagnosis of cardiac amyloidosis.

关 键 词：心肌疾病 淀粉样变性 磁共振成像 

分 类 号：R686[医药卫生—骨科学]