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