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Aortic Stenosis and Cardiac Amyloidosis: JACC Review Topic of the Week
Julien Ternacle1, Laura Krapf2, Dania Mohty3
1Institut Universitaire de Cardiologie et de Pneumologie de Québec/Québec Heart and Lung Institute, Université Laval, Québec City, Québec, Canada; Referral Center for Cardiac Amyloidosis, Mondor Amyloidosis Network, GRC Amyloid Research Institute and Cardiology Department, APHP Henri Mondor Hospital, Créteil, France; INSERM Unit U955, Team 8, Paris-Est Creteil University, Val-de-Marne, Créteil, France.
Insights
Calcific aortic stenosis (AS) and cardiac amyloidosis (CA) often coexist in older adults. Identifying CA in AS patients is crucial for guiding treatment and improving outcomes.
Area of Science:
- Cardiology
- Geriatrics
- Medical Diagnostics
Background:
- Calcific aortic stenosis (AS) and cardiac amyloidosis (CA) are age-related conditions that frequently overlap in elderly patients.
- Diagnosing CA in AS patients is challenging due to shared clinical and echocardiographic features.
- CA is associated with worse outcomes in AS patients, including heart failure, mortality, and reduced benefit from aortic valve replacement.
Purpose of the Study:
- To highlight the diagnostic challenges and clinical significance of concomitant cardiac amyloidosis in patients with calcific aortic stenosis.
- To emphasize the importance of accurate CA diagnosis for guiding AS management and treatment selection.
Main Methods:
- Review of current literature on the association between AS and CA.
- Analysis of diagnostic criteria and challenges for identifying CA in AS patients.
- Discussion of treatment implications, including surgical and transcatheter interventions.
Main Results:
- An estimated 5-15% of AS patients and up to 30% of those with low-flow, low-gradient AS may have underlying CA.
- Concomitant CA increases the risk of heart failure, mortality, and treatment futility with aortic valve replacement.
- Accurate diagnosis of CA is critical for appropriate management of AS and consideration of specific therapies for transthyretin amyloidosis.
Conclusions:
- Early and accurate diagnosis of CA in AS patients is essential for optimizing therapeutic strategies.
- Transcatheter aortic valve replacement may be a preferred option over surgical AVR in patients with concomitant AS and CA due to high surgical risk.
Abstract:
The prevalence of calcific aortic stenosis (AS) and of cardiac amyloidosis (CA) increases with age, and their association is not uncommon in the elderly. The identification of CA is particularly challenging in patients with AS because these 2 conditions share several features. It is estimated that ≤15% of the AS population and ≤30% of the subset with low-flow, low-gradient pattern may have CA. In patients with AS, CA is associated with increased risk of heart failure, mortality, and treatment futility with aortic valve replacement. In case of suspicion of CA, it is thus crucial to confirm the diagnosis to guide therapeutic management of AS and eventually implement recently developed pharmacological treatment dedicated to transthyretin amyloidosis. Given the high surgical risk of patients with AS and concomitant CA, transcatheter aortic valve replacement may be preferred to surgery in these patients.
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Aortic Regurgitation I: Introduction
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Cardiomyopathy II: Dilated Cardiomyopathy
Cardiomyopathy III: Hypertrophic Cardiomyopathy
Cardiomyopathy IV: Restrictive Cardiomyopathy

