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Aortic Valve Stenosis and Cardiac Amyloidosis: A Misleading Association
Andrea Bonelli1, Sara Paris1, Matilde Nardi1
1Cardiology Unit, Spedali Civili and University of Brescia, 25100 Brescia, Italy.
Insights
Cardiac amyloidosis (CA), especially transthyretin (ATTR)-CA, is often found with aortic stenosis (AS). Early diagnosis using non-invasive methods is crucial for managing these intertwined conditions.
Area of Science:
- Cardiology
- Cardiovascular Imaging
- Amyloidosis Research
Background:
- The co-occurrence of aortic stenosis (AS) and cardiac amyloidosis (CA) is more common than previously recognized.
- Transthyretin cardiac amyloidosis (ATTR-CA) is the predominant form of CA in patients with AS, frequently affecting elderly individuals.
Purpose of the Study:
- To review the shared pathophysiological characteristics, clinical presentations, and imaging findings of AS and CA.
- To highlight the diagnostic challenges and the importance of early detection of the AS-CA association.
Main Methods:
- Discussion of multiparametric evaluation strategies for detecting concurrent AS and CA.
- Emphasis on the role of modern non-invasive techniques, such as bone scintigraphy, for early CA diagnosis.
Main Results:
- ATTR-CA is the most prevalent type of CA in patients with AS.
- Accurate non-invasive imaging techniques facilitate early diagnosis of CA.
- Flow-charts are proposed to aid clinicians in diagnosing CA.
Conclusions:
- The combination of AS and CA presents diagnostic challenges due to overlapping features.
- Early identification of AS-CA is critical, particularly with emerging specific treatments for ATTR-CA.
- Understanding the AS-CA association impacts therapeutic decision-making for AS management.
Abstract:
The association between aortic stenosis (AS) and cardiac amyloidosis (CA) is more frequent than expected. Albeit rare, CA, particularly the transthyretin (ATTR) form, is commonly found in elderly people. ATTR-CA is also the most prevalent form in patients with AS. These conditions share pathophysiological, clinical and imaging findings, making the diagnostic process very challenging. To date, a multiparametric evaluation is suggested in order to detect patients with both AS and CA and choose the best therapeutic option. Given the accuracy of modern non-invasive techniques (i.e., bone scintigraphy), early diagnosis of CA is possible. Flow-charts with the main CA findings which may help clinicians in the diagnostic process have been proposed. The prognostic impact of the combination of AS and CA is not fully known; however, new available specific treatments of ATTR-CA have changed the natural history of the disease and have some impact on the decision-making process for the management of AS. Hence the relevance of detecting these two conditions when simultaneously present. The specific features helping the detection of AS-CA association are discussed in this review, focusing on the shared pathophysiological characteristics and the common clinical and imaging hallmarks.
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