Nine patients with cardiac amyloidosis were studied by echocardiography and the échocardiographie
abnormalities were then correlated with postmortem studies. All had congestive heart failure and autopsy-proven
amyloidosis. M-mode features included; (1) small or normal left-ventricular (LV) dimensions; (2) thickened interventricular
septa and LV posterior walls (89%); (3) left atrial enlargement (89%), and (4) reduced LV distensibility
(78%, 100%) and contractility (22%, 44%). Serial M-mode echocardiography revealed that cardiac amylodosis was
manifested initially more as diastolic than as systolic LV failure. The final stages in this disease were marked by
severe impairment of both systolic and diastolic LV functions. Two-dimensional echocardiography provided additional
features: (1) better appreciation of pericardial effusion (67%), and (2) a characteristic ‘granular sparkling’
appearance of ventricular walls (55%). These hyperrefractile myocardial echoes corresponded to histopathologically
scattered amyloid mass deposits in 4 out of 5 cases. There was no relation between the amyloid deposit type and a
hyperrefractile myocardial echo. Thus, cardiac involvement in systemic amyloidosis could be assessed noninvasively
by M-mode and two-dimensional echocardiography.