August 13, 2017European Heart Journal
Dangerous relationships: aortic stenosis and transthyretin cardiac amyloidosis
Key result
This editorial discusses the increasing prevalence and association of transthyretin cardiac amyloidosis in elderly patients with severe aortic stenosis undergoing transcatheter aortic valve replacement.
Key Points
- Examine the clinical intersection, pathophysiology, and diagnostic significance of transthyretin cardiac amyloidosis in elderly individuals with severe aortic stenosis.
- Synthesized epidemiological, clinical, and pathological evidence concerning aortic stenosis progression and transthyretin amyloid deposition.
- Evaluated diagnostic challenges and disease overlap among elderly patients eligible for transcatheter or surgical aortic valve replacement.
- Aortic stenosis prevalence rises to 9.8% in individuals aged 80 to 89 years, resulting in tens of thousands of global valve replacements annually.
- Transthyretin amyloidosis shows an autopsy prevalence of up to 25% among octogenarians, causing myocardial stiffening, diastolic dysfunction, arrhythmias, and progressive heart failure.
- Invasive and pathological evaluations show severe aortic stenosis and transthyretin cardiac amyloidosis frequently coexist in older populations, creating additive cardiac risks.
PICO
P
Population
Aortic stenosis and transthyretin cardiac amyloidosis
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Key expert perspectives
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“TAVR has offered up opportunities to improve aortic stenosis without open cardiac surgery, and part of the consequence of that is we were treating much older patients. We're now looking at octogenarians. If amyloid was present in 6% of average 75-year-olds, we would expect it to be higher [in older TAVR patients].”
“The three studies are 'extremely relevant' as they highlight just how common cardiac amyloidosis is in older patients with aortic stenosis. Their work shows that not only is amyloidosis prevalent but also clinical outcomes are poor, including in those undergoing valve replacement.”
ACAdam CastañoCardiologist, Columbia University Medical Center
“16% had evidence of cardiac amyloidosis”
Implication
This editorial highlights the increasingly recognized association between severe aortic stenosis and transthyretin cardiac amyloidosis in the elderly population.
Abstract
This editorial refers to ‘Unveiling transthyretin cardiac amyloidosis and its predictors among elderly patients with severe aortic stenosis undergoing transcatheter aortic valve replacement’†, by A. Castaño et al., on page 2879. Aortic stenosis (AS) is more commonly caused by calcification of a congenitally bicuspid or trileaflet valve. The prevalence of AS increases with age, averaging 0.2% in the 50- to 59-year cohort and increasing to 9.8% in the 80- to 89-year cohort.1 Because of low efficacy of medical therapy, annually worldwide 27 000 patients become eligible for transcatheter aortic valve replacement (TAVR) and 67 500 for surgical aortic valve replacement (AVR),1 according current guidelines.2 Transthyretin (TTR) amyloidosis (ATTR) is a fatal disorder that is characterized primarily by progressive neuropathy and cardiomyopathy. It occurs either as a mutant form (with autosomal dominant inheritance) or as a more frequent wild-type form (with predominant cardiac involvement).3 TTR, that is synthesized by the liver, has a physiological function of transporting thyroxin and retinol-binding proteins; it has a crucial role in behaviour, cognition, nerve regeneration, and axonal growth. More than 80 single point mutations can increase the likelihood of TTR to aggregate into insoluble amyloid fibres. Deposition of amyloid in the myocardial wall causes diastolic dysfunction, restrictive physiology with late loss of systolic function, arrhythmias, and heart failure.3 ATTR-cardiac amyloidosis (CA) has a male preponderance and was formerly known as ‘senile amyloidosis’, reflecting its first appearance beyond 60–70 years of age and prevalence at autopsy of up to 25% among octogenarians.4 Because of ageing of the population, the association between severe AS and ATTR-CA is more frequent than thought, as suggested by small previous studies, assessing invasively the presence of cardiac TTR deposition in elderly patients with severe symptomatic AS.5 , 6
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Cite This Study
Gargiulo et al. (2017) conducted an editorial in Aortic stenosis and transthyretin cardiac amyloidosis. This editorial discusses the increasing prevalence and association of transthyretin cardiac amyloidosis in elderly patients with severe aortic stenosis undergoing transcatheter aortic valve replacement.