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Published on: September 28, 2015
[Transthyretin Amyloid Cardiomyopathy as the Cause of Aortic Stenosis]
M V Ezhikova1, S N Nasonova1, I V Zhirov1
1Chazov Russian Cardiology Research and Production Complex, Moscow.
Insights
Diagnosing amyloid cardiomyopathy (ACM) is challenging due to varied symptoms like heart failure and valve issues. Differentiating amyloid-related aortic stenosis (AS) from degenerative AS is crucial, as amyloid causes 4-16% of AS in older adults.
Area of Science:
- Cardiology
- Cardiovascular Diseases
- Amyloidosis
Background:
- Amyloid cardiomyopathy (ACM) diagnosis presents significant challenges for clinicians.
- Diverse clinical presentations, including heart failure with preserved ejection fraction and hypertrophic phenotypes, complicate ACM diagnosis.
- Valvular complications, such as mitral/tricuspid insufficiency and aortic stenosis (AS), are common in ACM.
Purpose of the Study:
- To highlight the diagnostic difficulties in amyloid cardiomyopathy (ACM).
- To emphasize the importance of differentiating amyloid-origin aortic stenosis (AS) from degenerative AS.
- To underscore the prevalence of amyloid as a cause of AS in older populations.
Main Methods:
- Review of clinical manifestations of ACM.
- Analysis of common valvular involvement in ACM, specifically aortic stenosis.
- Literature review on the incidence of amyloidogenic AS in elderly patients.
Main Results:
- ACM diagnosis is hindered by a wide spectrum of symptoms.
- Aortic stenosis (AS) is a frequent valvular manifestation in ACM.
- Amyloidosis accounts for 4-16% of AS cases in patients aged 65 and older.
Conclusions:
- Accurate diagnosis of ACM requires awareness of its varied presentations.
- Distinguishing amyloid AS from degenerative AS is critical for appropriate patient management.
- Amyloidosis is an underrecognized cause of aortic stenosis in the elderly, necessitating specific diagnostic considerations.
Abstract:
Currently, the diagnosis of amyloid cardiomyopathy (ACM) causes great difficulties both for practicing primary care physicians and specialists. One of the reasons is the variety of symptoms in patients with ACM. The most common ACM manifestations include heart failure with preserved left ventricular ejection fraction, hypertrophic phenotype of cardiomyopathy, thromboembolic complications, and valvular apparatus damage. Common valvular manifestations are mitral and tricuspid valve insufficiency and aortic stenosis (AS). Diagnosis is particularly difficult in patients with AS requiring differentiation between AS as a degenerative defect and AS of amyloid origin. According to the literature, in 4-16% of cases of older patients (≥65 years), the genesis of AS is precisely the amyloid damage.
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