Amyloid Cardiomyopathy
Conditions
Keywords
cardiac amyloidosis, radiotherapy
Brief summary
Cardiac amyloidosis is responsible for significant morbidity associated with heart failure, and carries a poor prognosis. Currently there are very limited treatment options for this condition. Radiotherapy has been used successfully to treat amyloidosis elsewhere in the body, however has not been tried in cardiac amyloidosis. Therefore this study aims to assess the effect of radiotherapy on cardiac amyloidosis, to evaluate whether it can successfully reduce the burden of amyloid deposits in the myocardium as assessed by 18F-Amyloid PET.
Detailed description
The intervention will involve administration of external beam radiotherapy (5 fractions of 2Gy) focused to the heart. Measurements of effect will be assessed at 12 weeks by: Amyloid PET Cardiac MRI with administration of gadolinium and ultrasound A blood venous sample (cardiac biomarkers) Quality of life assessments
Interventions
RADIATIONlow dose radiotherapy
10 Gy in 5 fractions of 2 Gy on 5 consecutive days
Sponsors
Philippe Meyer
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
TREATMENT
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
65 Years to No maximum
Healthy volunteers
No
Inclusion criteria
* Age \>65 y.o. * Dyspnoea on exertion (NYHA II or more). * Stable elevated cardiac enzymes (ultra sensitive Troponin T \> 14 ng/L on consecutive sampling or BNP \> 100pg/mL) * A positive 99mTc-DPD cardiac scintigraphy (Grade 2 and 3) suggesting an ATTR or wt amyloidosis. * Additional imaging also compatible with cardiac amyloidosis (cardiac ultrasound showing basal to apical longitudinal strain gradient and magnetic resonance imaging with elevated T1 value or extracellular volume). * Compliance with the informed consent as attested by its signature. * Positive baseline 18F-Florbetapir imaging, as assessed visually and quantitatively by a Tissue to Background Ratio \> 1.45
Exclusion criteria
* Positive serum protein immunoelectrophoresis with monoclonal gammapathy. * Previous external beam radiotherapy including the chest. * Claustrophobia * Presence of internal non-MR compatible devices * Creatinine glomerular filtration rate \< 30 ml/min * Oncologic disease (excluding skin cancer) active or in remission from less than 5 years
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Assessment of the degree of amyloid | 12 weeks | Assess intra-individual change in a quantitative measure of amyloid deposits on 18F-Amyloid (Standard Uptake Value ratio) SUVR between amyloid PET scans before and after low dose RT |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Safety and adverse event associated with cardiac low dose RT | 6 months | Assess the number of patients who report adverse events |
| Modification in echocardiographic global longitudinal strain | 6 months | Assess intra-individual change in a quantitative measures |
| Modification in MRI extracellular volumes and T1 values | 12 weeks | Assess intra-individual change in a quantitative measures |
| Modification in cardiac biomarker (ultrasensitive troponin T and BNP blood levels) features of cardiac amyloidosis | 6 months | Assess intra-individual change in a quantitative measures |
| Modification of quality of life | 6 months | The quality of life will be assessed by the SF-36 short form health survey quality of life scale |
Countries
Switzerland
Contacts
Primary ContactRené Nkoulou, Dr.
Backup ContactPhilippe Meyer, Dr.
Outcome results
None listed