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.

Agency
National Institute of Health (NIH)
Institute
National Center for Research Resources (NCRR)
Type
General Clinical Research Centers Program (M01)
Project #
5M01RR000533-32
Application #
6409754
Study Section
National Center for Research Resources Initial Review Group (RIRG)
Project Start
1978-12-01
Project End
2001-11-30
Budget Start
Budget End
Support Year
32
Fiscal Year
2000
Total Cost
Indirect Cost
Name
Boston University
Department
Type
DUNS #
604483045
City
Boston
State
MA
Country
United States
Zip Code
02118
Rhee, Rennie L; Davis, John C; Ding, Linna et al. (2018) The Utility of Urinalysis in Determining the Risk of Renal Relapse in ANCA-Associated Vasculitis. Clin J Am Soc Nephrol 13:251-257
Liebschutz, Jane M; Buchanan-Howland, Kathryn; Chen, Clara A et al. (2018) Childhood Trauma Questionnaire (CTQ) correlations with prospective violence assessment in a longitudinal cohort. Psychol Assess 30:841-845
Guan, Yue; Roter, Debra L; Wolff, Jennifer L et al. (2018) The impact of genetic counselors' use of facilitative strategies on cognitive and emotional processing of genetic risk disclosure for Alzheimer's disease. Patient Educ Couns 101:817-823
Altman, Matthew C; Whalen, Elizabeth; Togias, Alkis et al. (2018) Allergen-induced activation of natural killer cells represents an early-life immune response in the development of allergic asthma. J Allergy Clin Immunol 142:1856-1866
Berti, Alvise; Warner, Roscoe; Johnson, Kent et al. (2018) Brief Report: Circulating Cytokine Profiles and Antineutrophil Cytoplasmic Antibody Specificity in Patients With Antineutrophil Cytoplasmic Antibody-Associated Vasculitis. Arthritis Rheumatol 70:1114-1121
Christensen, Kurt D; Uhlmann, Wendy R; Roberts, J Scott et al. (2018) A randomized controlled trial of disclosing genetic risk information for Alzheimer disease via telephone. Genet Med 20:132-141
Kattan, Meyer; Bacharier, Leonard B; O'Connor, George T et al. (2018) Spirometry and Impulse Oscillometry in Preschool Children: Acceptability and Relationship to Maternal Smoking in Pregnancy. J Allergy Clin Immunol Pract 6:1596-1603.e6
Wallace, Zachary S; Miloslavsky, Eli M; Cascino, Matthew et al. (2017) Effect of Disease Activity, Glucocorticoid Exposure, and Rituximab on Body Composition During Induction Treatment of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis. Arthritis Care Res (Hoboken) 69:1004-1010
Gern, James E; Calatroni, Agustin; Jaffee, Katy F et al. (2017) Patterns of immune development in urban preschoolers with recurrent wheeze and/or atopy. J Allergy Clin Immunol 140:836-844.e7
Guan, Yue; Roter, Debra L; Erby, Lori H et al. (2017) Disclosing genetic risk of Alzheimer's disease to cognitively impaired patients and visit companions: Findings from the REVEAL Study. Patient Educ Couns 100:927-935

Showing the most recent 10 out of 476 publications