J Nucl Cardiol. 2026 May;59S:102727. doi: 10.1016/j.nuclcard.2026.102727. Epub 2026 May 15.
ABSTRACT
Cardiac amyloidosis (CA) is characterized by cardiac amyloid fibril deposition leading to organ dysfunction. The two most common forms are transthyretin (TTR) amyloid cardiomyopathy (ATTR-CM) and immunoglobulin light chain cardiomyopathy (AL-CM). Treatment of CA includes disease-modifying therapies and treating symptoms and cardiac co-morbidities. Patients with heart failure and CA are managed with diuretics for volume optimization, but other guideline-directed medical therapies should be used on a case-by-case basis. Current disease-modifying therapies for ATTR-CM include TTR tetramer stabilizers and gene silencers. Investigational therapies include Clustered Regularly Interspaced Short Palindromic Repeats and associated Cas9 endonuclease (CRISPR-Cas9) systems and amyloid depleters. Current disease-modifying therapies for AL-CM include anti-plasma cell dyscrasia therapies. Investigational therapies include stabilizers and depleters. Nuclear scintigraphy is used to differentiate CA sub-type with no established role for nuclear imaging in therapeutic monitoring to date. However, given nuclear imaging and radiotracer advances, further research is being conducted regarding a nuclear imaging-based approach to assess amyloid burden and organ response to therapy.
PMID:42142948 | DOI:10.1016/j.nuclcard.2026.102727