Journal of Atherosclerosis and Thrombosis
Online ISSN : 1880-3873
Print ISSN : 1340-3478
ISSN-L : 1340-3478
Original Article
Unexpectedly High Prevalence of Acquired von Willebrand Syndrome in Patients with Severe Aortic Stenosis as Evaluated with a Novel Large Multimer Index
Toshihiro Tamura, Hisanori Horiuchi, Masao Imai, Tomohisa Tada, Hiroki Shiomi, Maiko Kuroda, Shunsuke Nishimura, Yusuke Takahashi, Yusuke Yoshikawa, Akira Tsujimura, Masashi Amano, Yukiko Hayama, Sari Imamura, Naoaki Onishi, Yodo Tamaki, Soichiro Enomoto, Makoto Miyake, Hirokazu Kondo, Kazuaki Kaitani, Chisato Izumi, Takeshi Kimura, Yoshihisa Nakagawa
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Keywords: Aortic stenosis, Gastrointestinal bleeding, Heyde's syndrome, Acquired von Willebrand syndrome, Large vWF multimer index
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2015 Volume 22 Issue 11 Pages 1115-1123

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Abstract

Aim: Severe gastrointestinal bleeding sometimes occurs in patients with aortic stenosis (AS), known as Heyde's syndrome. This syndrome is thought to be caused by acquired von Willebrand syndrome and is characterized by reduced large von Willebrand factor (vWF) multimers. However, the relationship between the severity of AS and loss of large vWF multimers is unclear.
Methods: We examined 31 consecutive patients with severe AS. Quantitative evaluation for loss of large vWF multimers was performed using the conventional large vWF ratio and novel large vWF multimer index. This novel index was defined as the ratio of large multimers of patients to those of controls.
Results: Loss of large vWF multimers, defined as the large vWF multimer index ＜80%, was detected in 21 patients (67.7%). The large vWF multimer ratio and the large vWF multimer index were inversely correlated with the peak aortic gradient (R=-0.58, p=0.0007, and R=-0.64, p＜0.0001, respectively). Anemia defined as hemoglobin ＜9.0 g/dl was observed in 12 patients (38.7%), who were regarded as Heyde's syndrome. Aortic valve replacement was performed in 7 of these patients, resulting in the improvement of anemia in all patients from a hemoglobin concentration of 7.5±1.0g/dl preoperatively to 12.4±1.3 g/dl postoperatively (p＜0.0001).
Conclusions: Acquired von Willebrand syndrome may be a differential diagnosis in patients with AS with anemia. The prevalence of AS-associated acquired von Willebrand syndrome is higher than anticipated.

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この記事はクリエイティブ・コモンズ [表示 - 非営利 - 継承 4.0 国際]ライセンスの下に提供されています。
https://creativecommons.org/licenses/by-nc-sa/4.0/deed.ja
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