Molecular Imaging of Primary Amyloid Cardiomyopathy
Brigham and Women's Hospital
Summary
Cardiac amyloidosis is a major cause of early treatment-related death and poor overall survival in individuals with systemic light chain amyloidosis. This project will develop a novel approach to visualize cardiac amyloid deposits using advanced imaging methods. The long-term goal of this work is to identify the mechanisms of cardiac dysfunction, in order to guide the development of novel life-saving treatments.
Description
Primary light chain amyloidosis (AL) is the most common systemic amyloidosis, resulting from a plasma cell dyscrasia, a hematological malignancy. It causes a restrictive cardiomyopathy (AL-CMP) in over 70% of individuals. AL-CMP is as lethal as stage 4 lung cancer and more lethal than any other form of restrictive heart disease; if untreated, the mortality rate is 50% within 18 months. Moreover, myocardial dysfunction, the hallmark of AL-CMP, significantly increases early treatment related mortality, predominantly cardiovascular death, and is a powerful predictor of poor long-term survival. Tw…
Eligibility
- Age range
- 18–99 years
- Sex
- All
- Healthy volunteers
- No
Inclusion criteria: * Age \> 18 years * Diagnosis of light chain amyloidosis by standard criteria (immunofixation of serum and urine, IgG free light chain (FLC) assay, a biopsy of fat pad/ bone marrow, or organ biopsy, followed by typing of the light chain using immunohistochemistry or immunogold assay with confirmation by Mass spectroscopy as needed) * For subjects traveling from out of town referred for systemic AL therapy based on clinical evaluation and laboratory testing, but, pending biopsy results, study enrollment and procedures may begin before official confirmation of biopsy resu…
Interventions
- RadiationF-18 florbetapir/C-11 acetate PET
F-18 florbetapir PET scan, C-11 acetate PET scan
- DeviceMRI
Cardiac MRI with gadolinium contrast.
- RadiationN-13 ammonia PET
N-13 ammonia PET scan following supine bicycle stress.
Location
- Brigham and Womens' HospitalBoston, Massachusetts