Aortic valve narrowing (aortic stenosis), the narrowing of the valve between the heart’s left pumping chamber and the main artery, is often accompanied by cardiac amyloidosis, where misfolded proteins are deposited in the heart muscle. These conditions often occur together in older people and carry a high risk of death.
Until now, treatment has consisted of valve replacement, while the deposits in the heart muscle remain untreated.
The new study, published in the European Heart Journal, found that, for patients with both conditions, combined treatment consisting of heart valve replacement and the amyloidosis drug tamafidis was linked to a lower risk of death.
The international research team looked at data from 226 patients with aortic stenosis and concomitant cardiac amyloidosis from ten countries.
Co-lead author Dr Thomas Treibel (UCL Institute of Cardiovascular Science) said: "Our study is the first to show benefit of amyloid therapy in patients with aortic valve stenosis and cardiac amyloidosis. This is an important finding because this patient group was previously not included in studies of amyloidosis therapy."
Study leader Dr Christian Nitsche, of MedUni Vienna, said: "Our results even show that patients with both conditions who received valve replacement and specific amyloidosis therapy had similar long-term survival rates to people with aortic stenosis without amyloidosis."
Targeted tests necessary
Both aortic stenosis and cardiac amyloidosis impair the heart’s pumping function and can lead to death if left untreated. Targeted therapy can slow the progression of amyloidosis, while valve replacement treats the mechanical stress caused by the narrowed heart valve. Around ten percent of patients with aortic stenosis also have amyloidosis, but this is often not diagnosed in everyday clinical practice.
Dr Nitsche added: "Our findings also suggest that patients with severe aortic valve stenosis should be screened for amyloidosis so that we can offer them targeted life-prolonging treatment options."
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Mark Greaves
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