Echocardiographic findings in nine patients with cardiac amyloidosis: their correlation with necropsy findings.

The echocardiographic abnormalities of nine patients with cardiac amyloidosis were correlated with their postmortem findings. All had congestive heart failure and autopsy-proven amyloidosis. M-mode echocardiographic features included (1) small or normal left ventricular (LV) dimensions; (2) thickened interventricular septa and posterior LV 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 amyloidosis was initially manifested as diastolic LV dysfunction rather than systolic dysfunction. The final stage of this disease was characterized by severe impairment of both systolic and diastolic LV functions. Two-dimensional echocardiography provided additional features: (1) a more accurate diagnosis of pericardial effusion (67%) and (2) a characteristic "granular sparkling" appearance of the ventricular wall (55%). In four of five cases, these hyperrefractile myocardial echoes corresponded to scattered amyloid deposits histopathologically. There was no correlation between the types of amyloid deposits and the hyperrefractile myocardial echoes. Thus, cardiac involvement in systemic amyloidosis could be non-invasively assessed by M-mode and two-dimensional echocardiography.