Impact of disease-modifying therapies on imaging parameters in cardiac amyloidosis: A systematic review and meta-analysis.

Transthyretin-mediated (ATTR) and immunoglobulin light-chain (AL) cardiac amyloidosis causes progressive myocardial dysfunction and poor prognosis. Disease-specific therapies in ATTR and anti-clonal therapies in AL cardiac amyloidosis may modify disease, but treatment-induced changes in prognostic cardiac imaging markers remain incompletely defined. We systematically searched PubMed/MEDLINE, Scopus, CINAHL, Web of Science, and EMBASE for studies reporting treatment-associated changes in global longitudinal strain (GLS), left ventricular (LV) wall thickness, and extracellular volume (ECV) assessed by echocardiography or cardiac magnetic resonance. Random-effects meta-analyses pooled mean changes. Seventeen studies met inclusion criteria. In ATTR cardiac amyloidosis, therapy with silencers or RNA-depleting agents was associated with improved GLS (pooled mean difference [MD] -0.97 %, 95 % CI -1.27 to -0.68) and reduced LV wall thickness (MD -0.72 mm, 95 % CI -1.26 to -0.17) versus no therapy, while ECV showed no significant change (MD -1.93 %, 95 % CI -15.27 to 11.41). In AL amyloidosis, complete hematologic response was linked to greater improvement than non-response in GLS (MD -1.19 %, 95 % CI -2.20 to -0.17) and LV wall thickness (MD -0.87 mm, 95 % CI -1.31 to -0.43). NMA in ATTR cardiac amyloidosis showed that patisiran (MD -1.04 %, 95 % CI -1.56 to -0.52), tafamidis (-0.91 %, -1.40 to -0.41), and vutrisiran (-0.90 %, -1.75 to -0.05) improved GLS versus placebo, compared to acoramidis. Disease-directed therapy in ATTR and hematologic response in AL cardiac amyloidosis are associated with preservation or improvement of GLS and LV wall thickness, supporting their use as surrogate endpoints for treatment monitoring.