Uchida Tetsuro, Hamasaki Azumi, Ohba Eiichi, Yamashita Atsushi, Hayashi Jun, Sadahiro Mitsuaki
Second Department of Surgery, Faculty of Medicine, Yamagata University, 2-2-2 Iida-Nishi, Yamagata, 990-9585, Japan.
J Cardiothorac Surg. 2017 Aug 8;12(1):65. doi: 10.1186/s13019-017-0629-x.
Heyde syndrome is known as a triad of calcific aortic stenosis, anemia due to gastrointestinal bleeding from angiodysplasia, and acquired type 2A von Willebrand disease. This acquired hemorrhagic disorder is characterized by the loss of the large von Willebrand factor multimers due to the shear stress across the diseased aortic valve. The most frequently observed type of bleeding in these patients is mucosal or skin bleeding, such as epistaxis, followed by gastrointestinal bleeding. On the other hand, intracranial hemorrhage complicating Heyde syndrome is extremely rare.
A 77-year-old woman presented to our hospital with severe aortic stenosis and severe anemia due to gastrointestinal bleeding and was diagnosed with Heyde syndrome. Although aortic valve replacement was performed without recurrent gastrointestinal bleeding, postoperative life-threatening acute subdural hematoma occurred with a marked midline shift. Despite prompt surgical evacuation of the hematoma, she did not recover consciousness and she died 1 month after the operation.
Postoperative subdural hematoma is rare, but it should be kept in mind as a devastating hemorrhagic complication, especially in patients with Heyde syndrome.
海德综合征被认为是由钙化性主动脉瓣狭窄、血管发育异常导致的胃肠道出血引起的贫血以及获得性2A型血管性血友病组成的三联征。这种获得性出血性疾病的特征是由于病变主动脉瓣上的剪切应力导致大的血管性血友病因子多聚体丢失。这些患者最常出现的出血类型是黏膜或皮肤出血，如鼻出血，其次是胃肠道出血。另一方面，海德综合征并发颅内出血极为罕见。
一名77岁女性因严重主动脉瓣狭窄和胃肠道出血导致的严重贫血入住我院，被诊断为海德综合征。尽管进行了主动脉瓣置换术且未再发生胃肠道出血，但术后出现了危及生命的急性硬膜下血肿，并伴有明显的中线移位。尽管及时进行了血肿手术清除，但她仍未恢复意识，术后1个月死亡。
术后硬膜下血肿罕见，但应将其视为一种严重的出血性并发症，尤其是在海德综合征患者中。
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