The association between AS and GI bleeding was first described by Heyde in 1958 [
1]. In 1992, a combination of AS and recurrent GI bleeding from angiodysplasia was defined as Heyde syndrome [
2]. Although the exact pathophysiology of the syndrome is still unclear, a recent report by Vincentelli et al. showed that acquired type 2A von Willebrand disease causes Heyde syndrome [
3]. vWF synthesized by endothelial cells is stored as ultra-large multimers and mediates platelet adhesion for hemostasis. Degenerative AS is associated with increased destruction of high molecular weight multimers of vWF by the shear stress across the stenotic aortic valve. Degradation of the heaviest multimers of vWF, that can induce bleeding from intestinal angiodysplasia, is the basis for the coagulopathy in Heyde syndrome [
3]. Heyde syndrome refers to a triad of aortic stenosis, acquired coagulopathy (type 2A von Willebrand disease) and anemia due to bleeding from intestinal angiodysplasia or from an idiopathic site [
6]. All three criteria were positive in our patient.
Patients with Heyde syndrome who are treated by intestinal resection generally continue to bleed from other sites, while AVR usually cures the clotting disorder and the recurrent anemia [
6]. AVR, rather than bowel resection, was considered to lead to long-term resolution of congestive heart failure and anemia in severe cases.
Intracranial hemorrhagic events occurring following open-heart surgery are uncommon but may prove devastating due to enhanced bleeding tendency caused by heparin administration, hypothermia, and postoperative anticoagulation therapy [
7‐
10]. Of these, the incidence of subdural hematoma after open-heart surgery was 0.1% [
7]. Subdural hematomas result from tearing of bridging veins between the dura and cerebral hemispheres, usually resulting from apparently trivial head injury. Krous et al. described the cause of subdural hematoma after cardiac surgery as a tearing of bridging veins that occurs with a fluid shift to the brain and a bleeding tendency induced by heparin administration [
8]. Our patient had no history of head injury and a preoperative brain CT revealed no abnormalities. We suspected that the subdural hematoma may have been caused by rapid alterations in cerebral volume, leading to a tearing of the dural bridging veins under cardiopulmonary bypass. Once bleeding occurred, the subdural hematoma would rapidly enlarge owing to a bleeding tendency following postoperative consumptive coagulopathy and heparin administration. The hemorrhagic disorder associated with Heyde syndrome would enhance massive bleeding which resulted in the formation of the large subdural hematoma with severe midline shift. Subdural hematoma following open-heart surgery is sometimes life-threatening, but early diagnosis and prompt treatment may lead to neurological recovery in many cases [
7‐
10]. Especially in our patient, severe hemorrhagic tendency enhanced by Heyde syndrome led to large subdural hematoma and irreversible neurological deterioration. Unfortunately, her neurological state did not improve and she died 1 month after the cardiac operation. Pre-existing coagulopathy, including Heyde syndrome, in patients undergoing open-heart surgeries may cause prolonged bleeding from the surgical sites or unexpected organ bleeding because of the necessity for heparinization. In conclusion, if patients with AS show GI bleeding, Heyde syndrome should be suspected, and vWF multimers should be evaluated for diagnosis [
5]. Subdural hematoma subsequent to open-heart surgery is rare, but our experience may draw the attention of cardiac surgeons, not only to recurrent GI bleeding, but also to the possibility of acute subdural hematoma in patients with Heyde syndrome undergoing AVR. To our knowledge, this is the first report of life-threatening subdural hematoma complicating Heyde syndrome.