Transthyretin cardiac amyloidosis (ATTR-CM) is an infiltrative cardiomyopathy caused by amyloid fibril deposition, leading to heart failure and arrhythmias. Despite advances in diagnosis, the disease remains commonly unrecognized and presents heterogeneously. Recent therapies targeting transthyretin stabilization and gene silencing have improved outcomes, but current staging systems based on biological and functional markers have limited ability to guide treatment.
Imaging techniques such as cardiac magnetic resonance (CMR) provide tissue characterization, but noninvasive molecular imaging of myocardial fibrotic activity remains limited. Positron emission tomography (PET) tracers targeting fibroblast activation protein (FAPI), labeled with gallium-68 (68Ga), offer a promising approach to detect and quantify fibroblast activity associated with myocardial remodeling.
This study aims to evaluate \[68Ga\]Ga-FAPI PET imaging for staging ATTR-CM and distinguishing patients with disease progression under therapy. The investigators hypothesize that \[68Ga\]Ga-FAPI uptake reflects fibrotic activity correlating with disease severity and progression. If validated, \[68Ga\]Ga-FAPI PET could serve as a novel biomarker for improved staging and personalized strategies in ATTR-CM.
Men or women ≥ 18 years
Definite ATTR-CM diagnosis based on the 2021 ESC expert consensus
Written and signed informed consent (obtained on the screening day at the latest and before any investigation)
Affiliation with French social security system or beneficiary from such system
History of Myocardial infarction or myocarditis
Severe aortic stenosis
Current or prior participation in a clinical trial evaluating a TTR-targeted gene-silencing therapy (TTR= transthyretin) or an amyloid-depleting treatment for cardiac transthyretin amyloidosis (ATTR-CM).
Any psychological, familial, sociological or geographical condition potentially hampering compliance with the study protocol and follow-up schedule