The evolving landscape of screening, management and treatment strategies of aortic stenosis in ATTR amyloidosis.
Journal:
European journal of internal medicine
Published Date:
Nov 29, 2025
Abstract
Transthyretin amyloid cardiomyopathy (ATTR-CM) and aortic stenosis (AS) frequently coexist in elderly patients, particularly men, creating a complex clinical scenario with overlapping symptoms and imaging features. Both conditions are strongly age-dependent, and their association is increasingly recognized as more than coincidental. Transthyretin deposits have been identified within stenotic aortic valves, supporting the hypothesis that mechanical stress and shear forces may promote protein misfolding and amyloid deposition. The reported prevalence of ATTR-CM among patients with severe AS ranges from 4 % to 29 %, particularly in those undergoing transcatheter aortic valve replacement (TAVR). Diagnosis remains challenging and a multimodality imaging strategy integrating ECG-echocardiographic discordance, cardiac magnetic resonance with extracellular volume (ECV) quantification, and bone scintigraphy is essential to achieve final confirmation of disease. Newer tools such as the RAISE and GRAM scores and CT-derived myocardial extracellular volume (ECV) quantification enhance screening accuracy in this population. Clinically, even moderate AS may worsen restrictive hemodynamics in ATTR-CM, emphasizing the need for early recognition and individualized management. Contemporary studies suggest that carefully selected patients with dual pathology benefit from TAVR, achieving outcomes comparable to those with isolated AS, especially when patients are initiated on disease-modifying therapies. Future research should clarify the mechanistic link between valve and myocardial amyloid deposition, refine risk stratification, and integrate artificial intelligence to improve early detection and guide combined interventional and pharmacologic strategies.
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