Concomitant cardiac amyloidosis and aortic stenosis: update on diagnosis and management

Abstract

Concomitant aortic stenosis (AS) and cardiac amyloidosis (CA) represent a significant and increasingly recognized clinical challenge, particularly in elderly populations. This review aims to present current knowledge on the prevalence, clinical characteristics, imaging findings, outcomes and management strategies for patients with both AS and CA. Studies indicate that transthyretin cardiac amyloidosis (ATTR-CA) frequently coexists with AS, especially in patients undergoing transcatheter aortic valve replacement (TAVR), with prevalence rates ranging from 4% to 16%. The dual pathology exacerbates heart failure risk, increases mortality, and complicates therapeutic decision-making. Diagnosing CA in the presence of AS is complex due to overlapping clinical and imaging features. A multi-parametric diagnostic approach is essential, incorporating clinical assessment, advanced echocardiography, cardiac magnetic resonance imaging, and bone scintigraphy of the heart. The presence of CA influences the management of AS, often favoring TAVR over surgical valve replacement due to increased surgical risk. Emerging pharmacological treatments for ATTR-CA offer survival benefits and may alter the natural disease progression. This review highlights the need for heightened clinical awareness, early diagnosis through advanced imaging modalities, and tailored therapeutic strategies to improve outcomes in patients with concomitant AS and CA.