{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,4,24]],"date-time":"2025-04-24T04:24:17Z","timestamp":1745468657144,"version":"3.40.4"},"reference-count":30,"publisher":"S. Karger AG","license":[{"start":{"date-parts":[[2024,6,24]],"date-time":"2024-06-24T00:00:00Z","timestamp":1719187200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/karger.com\/pages\/terms-and-conditions"},{"start":{"date-parts":[[2024,6,24]],"date-time":"2024-06-24T00:00:00Z","timestamp":1719187200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/karger.com\/pages\/terms-and-conditions"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cardiology"],"abstract":"<jats:p>&lt;b&gt;&lt;i&gt;Introduction:&lt;\/i&gt;&lt;\/b&gt; High-molecular-weight (HMW) von Willebrand factor (VWF) multimer deficiency occurs in classical low-flow, low-gradient (LF\/LG) aortic stenosis (AS) due to shear force-induced proteolysis. The prognostic value of HMW VWF multimer deficiency is unknown. Therefore, we sought to evaluate the impact of HMW VWF multimer deficiency on clinical outcome. &lt;b&gt;&lt;i&gt;Methods:&lt;\/i&gt;&lt;\/b&gt; In this prospective research study, a total of 83 patients with classical LF\/LG AS were included. All patients underwent dobutamine stress echocardiography to distinguish true-severe (TS) from pseudo-severe (PS) classical LF\/LG AS. HMW VWF multimer ratio was calculated using densitometric Western blot band quantification. The primary endpoint was all-cause mortality. &lt;b&gt;&lt;i&gt;Results:&lt;\/i&gt;&lt;\/b&gt; Mean age was 79 \u00b1 9 years, and TS classical LF\/LG AS was diagnosed in 73% (&lt;i&gt;n&lt;\/i&gt; = 61) and PS classical LF\/LG AS in 27% (&lt;i&gt;n&lt;\/i&gt; = 22) of all patients. Forty-six patients underwent aortic valve replacement (AVR) and 37 were treated conservatively. During a mean follow-up of 27 \u00b1 17 months, 47 deaths occurred. Major bleeding complications after AVR (10\/46; 22%) were more common in patients with HMW VWF multimer ratio &amp;lt;1 (8\/17; 47%) in comparison to patients with a normal multimer pattern (2\/29; 7%) at baseline (&lt;i&gt;p&lt;\/i&gt; = 0.003). In a multivariable Cox regression analysis, HMW VWF multimer deficiency was a predictor of all-cause mortality (HR: 3.02 [95% CI: 1.31\u20136.96], &lt;i&gt;p&lt;\/i&gt; = 0.009) in the entire cohort. This association was driven by higher mortality rates in the AVR group (multivariable-adjusted HR: 9.4; 95% CI 2.0\u201343.4, &lt;i&gt;p&lt;\/i&gt; = 0.004). &lt;b&gt;&lt;i&gt;Conclusions:&lt;\/i&gt;&lt;\/b&gt; This is the first study to demonstrate the predictive value of HMW VWF multimer ratio for risk stratification in patients with classical LF\/LG AS. HMW VWF multimer deficiency was associated with an increased risk of all-cause mortality and major bleeding complications after AVR. <\/jats:p>","DOI":"10.1159\/000539731","type":"journal-article","created":{"date-parts":[[2024,7,22]],"date-time":"2024-07-22T21:15:29Z","timestamp":1721682929000},"page":"1-9","source":"Crossref","is-referenced-by-count":0,"title":["Prognostic Impact of High-Molecular-Weight von Willebrand Factor Multimer Ratio in Classical Low-Flow Low-Gradient Aortic Stenosis"],"prefix":"10.1159","author":[{"given":"Joerg","family":"Kellermair","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hermann","family":"Blessberger","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Helmut 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