Echocardiographie Findings in Nine Patients with Cardiac Amyloidosis

Abstract

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.

Evaluation and Management of the Cardiac Amyloidosis

“…Reduced systolic function, as measured by low ejection fraction or poor wall thickening, is uncommon until the more severe stages of disease and can be absent in up to 75% of cases (12,23,(25)(26)(27)33). Diastolic dysfunction is the hallmark, and may be present in all patients, though its identification can be difficult with standard techniques, with 21% to 88% of patients showing a restrictive pattern on Doppler mitral inflow assessment (25,35,36).…”

Heart Transplantation in a 68-Year-Old Patient with Senile Systemic Amyloidosis

“…Cardiac amyloid deposits can only be diagnosed by biopsy. Nevertheless, an echo that shows concentrically thickened ventricular walls with granular sparkling (ground glass) appearance, normal or small left ventricular cavity, thickened interventricular septa, enlarged atria and thickened valves is strongly suggestive of amyloidosis (3,8,9). Thus the diagnosis can reasonably be assumed, particularly when amyloid has been demonstrated in other sites, or when the patient has a recognized amyloidogenic mutation (3).…”

Amyloid Heart Disease

“…Echocardiography is a useful screening tool, and findings for patients with proven CA include concentric left ventricular hypertrophy, small to normal left ventricular diameters (>90 %), left atrial enlargement (89-91 %), a granular and sparkling appearance of the myocardium (55-82 %), moderate to large pericardial effusion (64-67 %), and left ventricular systolic dysfunction (44-73 %) ( Figure 2). 14,20 The transmitral flow velocity is often compatible with abnormal relaxation in all types of CA, and is seen in 57-64 % of patients, usually in the early phase of the disease. 13,14 Intracardiac thrombus has also been observed on echocardiograms of patients with AL CA.…”

“…14,20 The transmitral flow velocity is often compatible with abnormal relaxation in all types of CA, and is seen in 57-64 % of patients, usually in the early phase of the disease. 13,14 Intracardiac thrombus has also been observed on echocardiograms of patients with AL CA. 26 The majority of these findings, while more common in CA, are also seen in patients with hypertrophic cardiomyopathy (HCM).…”