Echocardiographic signs of aortic valve dysfunction in patients with Philadelphia-negative myeloproliferative neoplasms

Abstract

Abstract Background Patients with Philadelphia-negative myeloproliferative neoplasms (MPNs) have an increased prevalence of calcifications on the aortic valve, but the prevalence of aortic valve stenosis or regurgitation has only been reported in one small study based on outdated guidelines, and a few case reports. Chronic inflammation and increased shear stress on the aortic valve increase the risk of developing aortic valve dysfunction. The proliferation of mature blood cells in patients with MPNs leads to increased blood viscosity that increase shear stress on endothelial cells in the blood vessels, and likely also on the aortic valve. Furthermore, there is evidence of chronic inflammation in patients with MPNs. Purpose To investigate if patients with MPNs had a higher prevalence of aortic valve stenosis and/or regurgitation compared to the general population. Methods Patients with the MPN diagnosis were recruited between 2016 and 2018 and examined with echocardiography. The patients were matched 1:1 on sex and 5-year age span, with participants from CCHS, a large on-going population study. Aortic valve stenosis was defined as an AV Vmax >2.5 m/s measured by continuous wave Doppler and aortic valve regurgitation was defined as colour Doppler with a visible regurgitation jet. Results We included 167 patients with MPNs (mean age 66 years [SD ± 10]) and 167 controls (mean age 62 [SD ± 10]). There was a significantly higher median AV Vmax in the MPN cohort (1.4 vs 1.3 m/s, p<0.0001) and a significantly lower mean aortic valve area (2.2 vs 2.6 cm2, p<0.0001). There were nine patients (5%) in the MPN cohort who fulfilled the criterion for aortic valve stenosis while no one in the control group fulfilled this criterion (p=0.0035). Aortic valve regurgitation was found in 41 (25%) MPN patients and 8 (5%) controls (p<0.0001) Conclusion Patients with MPNs have a higher prevalence of aortic valve stenosis and aortic valve regurgitation compared to the general population.Graphical overviewTable 1