Clinical features and MRI characteristics in patients with cardiac amyloidosis
10.3760/cma.j.issn.1005-1201.2010.12.017
- VernacularTitle:心肌淀粉样变性的临床表现及MRI特征
- Author:
Junyi WAN
;
Shihua ZHAO
;
Shiliang JIANG
;
Yan ZHANG
;
Chaowu YAN
;
Minjie LU
;
Yang ZHOU
;
Jie HUANG
;
Hong ZHAO
- Publication Type:Journal Article
- Keywords:
Cardiomyopathies;
Amyloidosis;
Magnetic resonance imaging
- From:
Chinese Journal of Radiology
2010;44(12):1297-1299
- CountryChina
- Language:Chinese
-
Abstract:
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.
