Novel Imaging Tools in Newly-diagnosed Patients With Cardiac AL Amyloidosis

Study Overview
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Brief Summary
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This will be a systematic, combined, prospective assessment of the novel echographic, CMR, and PET imaging tools in newly-diagnosed patients with cardiac AL amyloidosis at baseline and after treatment.

Official Title
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Molecular, Magnetic Resonance, and Echocardiographic Imaging Combined With Biomarkers of Cardiac and Clonal Disease to Predict Survival and Assess Response to Therapy in Cardiac AL Amyloidosis

Conditions
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AL Amyloidosis

Intervention / Treatment
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* Drug: [18F]Florbetaben


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: age more than 18; histological diagnosis of AL amyloidosis; measurable cardiac involvement as per current response criteria (i.e. NT-proBNP >650 ng/L); measurable hematologic disease (dFLC >20 mg/L); adequate renal function (eGFR >30 mL/min) in order to be safely administered gadolinium; absence of atrial fibrillation with uncontrolled heart rate; absence of implantable cardiac devices; absence of pulmonary amyloidosis histologically documented; plan to start anti-plasma cell chemotherapy; plan to assess response at the Pavia center after 6 months; have given written informed consent to participate. Exclusion Criteria: non-AL amyloidosis; NYHA class IV; PS-ECOG >3; severe allergy to paramagnetic tracer; severe claustrophobia; pregnant or nursing women;

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Diagnostic
Intervention Model: Single Group Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: 18F-florbetaben PET-CT scans<br> | Drug: [18F]Florbetaben<br>* Patients will undergo on the same day: 18F-florbetaben PET-CT scans. The dose to be injected intravenously (6 second/mL) will be 370 MBq (for a 70 Kg patient); standard assessment of clonal and organ disease; echocardiography; cardiac magnetic resonance. All the patients will undergo those evaluations at baseline and 6 months after treatment initiation.<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Evaluation of the prognostic relevance of advanced imaging variables. | - for CMR: T1, T2, ECV, indexed volumes, mass, ejection fraction (EF); | 12 months after diagnosis | 
| Evaluation of the prognostic relevance of advanced imaging variables. | - for echocardiography: left ventricular wall thickness (mLVW), EF, 2D-GLS, midwall fractional shortening (mFS), and stroke volume index (SVI); | 12 months after diagnosis | 
| Evaluation of the prognostic relevance of advanced imaging variables. | - for F-PET: myocardial uptake score. | 12 months after diagnosis | 
| Evaluation of advanced imaging variables in response assessment. | The same variables considered at baseline will be measured 6 months after initiation of chemotherapy targeting the amyloid plasma cell clone. Changes in these variables compared to baseline will be considered. | 6 months after initiation of chemotherapy targeting the amyloid plasma cell clone | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Amyloidosis, Prognosis, [18F]Florbetaben