This protocol is designed to characterize the echocardiographic features of cardiac amyloidosis in patients with familial (AF) amyloidosis and AL amyloidosis and to assess whether current therapies designed to be curative for these 2 systemic diseases result in regression of cardiac amyloidosis, a frequent reason for death in such patients. Boston University Medical Center has the largest experience in the world with stem cell transplantation for AL amyloidosis and among the largest cohort of patients with familial amyloidosis, and thus we are uniquely qualifies to assess this problem. Patients will have echocardiograms when being evaluated for cardiac amyloidosis and then subsequently if they undergo liver transplantation (for familial amyloidosis) or stem cell transplantation (for AL amyloidosis). Serial echocardiograms will be preformed. Sophisticated measurements of global and regional rates of relaxation and contraction of the ventricle will be assessed using a new echocardiographic modality Doppler tissue imaging, with additional measurements with acoustic quantification and color M-mode imaging. Wall thickness and relative wall thickness of the left ventricle will be measured, left ventricle mass calculated, and measurements of right ventricle thickness and function will be assessed at each visit. As a secondary measure of ventricular function, Doppler filling properties, atrial size and volume will be measured and calculated.
Specific Aims : 1. To compare the echocardiographic features of AL amyloidosis and familial amyloidosis 2. To characterize the changes in cardiac structure and function following liver transplantation for familial amyloidosis and to assess whether liver transplantation is associated with regression/lack of progression of cardiac amyloidosis. 3. To characterize the cardiac geometric and functional changes in patients with AL amyloidosis treated with stem cell transplantation and to characterize the degree and rate of regression of cardiac involvement However, in addition many patients are screened for cardiac amyloidosis and by conventional measurements do not appear to have cardiac involvement. These patients may or may not receive stem cell or liver transplantation. In these patients, the new refined echocardiographic measurements will be added to standard echocardiograms to assess for subtle involvement of the heart. 4. In addition, all patients screened for amyloidosis will have regular echo with new parameters measured to evaluate the predictive value of abnormal myocardial relaxation as measured by Doppler tissue imaging for predicting the development of overt cardiac amyloidosis.
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