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 post- contrast 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. (authors)