心肌淀粉样变性的临床表现及MRI特征

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.

Clinical features and MRI characteristics in patients with cardiac amyloidosis

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.