Abstract 3167: Aortic Stenosis and Acquired Type 2a von Willebrand Syndrome: Correlation Between Severity of Aortic Stenosis and Gastrointestinal Angiodysplasia(Heyde’s Syndrome)
Background: Aortic stenosis(AS) complicated with gastrointestinal angiodysplasia (Heyde’s syndrome) is associated with acquired type 2A von Willebrand syndrome. However, the prevalence and the cause of hemostatic abnormality in AS are not widely recognized.
Methods: We investigated 12 consecutive patients who were diagnosed moderate to severe AS by echocardiography. We analyzed echocardiographic data and other clinical or biochemical parameters. To assess the significance of acquired type 2A von Willebrand syndrome in AS patients, association between severity of AS and serum levels of 3 subtypes of von Willebrand factor were analyzed.
Results: The median age was 77.8 years and 6 patients (50%) had anemia. Six patients had severe AS (max pressure gradient=105.9±11.6mmHg) and 6 patients had moderate AS (52.6±11.6mmHg). Three of 6 patients with severe AS had a history of gastrointestinal bleeding and a deficiency of high-molecular-weight multimers of von Willebrand factor, and were diagnosed as Heyde’s syndrome. Two of the 6 patients with moderate AS showed a deficiency of high-molecular-weight multimers of von Willebrand factor (i.e. the incidence was 50% in severe AS vs 33% in moderate AS). Mean pressure gradient(78.0±6.9mmHg vs 37.5±4.9mmHg, p=0.02) and activity of ADAMTS-13(91.5±23.7 vs 49.4±12.6, p=0.03) was significantly higher in AS complicated with VWS-2A. Because 2 of the 3 patients with Heyde’s syndrome suffered from acute gastrointestinal bleeding, they underwent colonoscopy and an angiodysplasia was revealed in the ascending colon of each patient. In the sera of the two Heyde’s syndrome patients who received valve replacement, von Willebrand factor-multimer profiles were normalized after surgery, and thereafter, no gastrointestinal bleeding has been documented in both patients(follow-up period was 1075 and 1313days).
Conclusions: The severity of AS and activity of ADAMTS-13 may be related to the acquired type 2A von Willebrand syndrome complicated with AS(Heyde’s syndrome). Deficiency of high-molecular-weight multimers of von Willebrand factor may be associated with an impaired hemostasis in severe AS, which was reversible by aortic valve replacement.