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A surgical case of aortic stenosis with recurrent gastrointestinal bleeding: Heyde syndrome.
Int J Surg Case Rep 2018; 53:281-284IJ

Abstract

INTRODUCTION

The combination of aortic stenosis, acquired coagulopathy, and anemia due to gastrointestinal (GI) bleeding is described as Heyde syndrome.

PRESENTATION OF CASE

We report a surgical case of a 77-year-old man who was admitted because of melena and exertional chest compression. GI endoscopy could not reveal the origin of the GI bleeding. Conservative therapy including fasting and transfusion improved the anemia. Echocardiography demonstrated severe aortic stenosis (AS) with a hypertrophied left ventricle. Hematologic examination by gel electrophoresis showed deficiency of high-molecular-weight multimers of von Willebrand factor (vWF), resulting in the diagnosis of Heyde syndrome. He electively underwent aortic valve replacement (AVR) with a bioprosthesis using standard cardiopulmonary bypass. In the perioperative period, the patient had no recurrent anemia. He was discharged without subjective symptoms on postoperative day 18. The 20-month follow-up was unremarkable, with no episode of recurrent GI bleeding.

DISCUSSION

Heyde syndrome is associated with acquired von Willebrand disease type IIA and AS. The true characteristic of von Willebrand disease type IIA is hemorrhagic diathesis caused by deficiency of high-molecular-weight multimers of vWF. Under the influence of high shear stress caused by AS, vWF is stretched and easily cleaved by vWF-cleaving protease. Consequently, it causes deficiency of high-molecular-weight multimers of vWF and primary hemostasis impairment. Therefore, the most effective treatment for Heyde syndrome is correction of AS. AVR can improve not only the hemodynamic status but also coagulopathy.

CONCLUSION

The differential diagnosis of patients with recurrent GI bleeding with concurrent systolic murmur should include Heyde syndrome.

Authors+Show Affiliations

Department of Cardiovascular Surgery, Oji General Hospital, Tomakomai, Hokkaido, Japan. Electronic address: miijima@ojihosp.or.jp.Department of Cardiovascular Surgery, Oji General Hospital, Tomakomai, Hokkaido, Japan.Department of Cardiovascular Surgery, Oji General Hospital, Tomakomai, Hokkaido, Japan.Department of Cardiovascular Surgery, Oji General Hospital, Tomakomai, Hokkaido, Japan.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

30448636

Citation

Iijima, Makoto, et al. "A Surgical Case of Aortic Stenosis With Recurrent Gastrointestinal Bleeding: Heyde Syndrome." International Journal of Surgery Case Reports, vol. 53, 2018, pp. 281-284.
Iijima M, Itoh N, Murase R, et al. A surgical case of aortic stenosis with recurrent gastrointestinal bleeding: Heyde syndrome. Int J Surg Case Rep. 2018;53:281-284.
Iijima, M., Itoh, N., Murase, R., & Makino, Y. (2018). A surgical case of aortic stenosis with recurrent gastrointestinal bleeding: Heyde syndrome. International Journal of Surgery Case Reports, 53, pp. 281-284. doi:10.1016/j.ijscr.2018.10.078.
Iijima M, et al. A Surgical Case of Aortic Stenosis With Recurrent Gastrointestinal Bleeding: Heyde Syndrome. Int J Surg Case Rep. 2018;53:281-284. PubMed PMID: 30448636.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - A surgical case of aortic stenosis with recurrent gastrointestinal bleeding: Heyde syndrome. AU - Iijima,Makoto, AU - Itoh,Nagomi, AU - Murase,Ryota, AU - Makino,Yutaka, Y1 - 2018/11/10/ PY - 2018/09/14/received PY - 2018/10/20/revised PY - 2018/10/25/accepted PY - 2018/11/19/pubmed PY - 2018/11/19/medline PY - 2018/11/19/entrez KW - Aortic stenosis KW - Case report KW - Gastrointestinal bleeding KW - Heyde syndrome SP - 281 EP - 284 JF - International journal of surgery case reports JO - Int J Surg Case Rep VL - 53 N2 - INTRODUCTION: The combination of aortic stenosis, acquired coagulopathy, and anemia due to gastrointestinal (GI) bleeding is described as Heyde syndrome. PRESENTATION OF CASE: We report a surgical case of a 77-year-old man who was admitted because of melena and exertional chest compression. GI endoscopy could not reveal the origin of the GI bleeding. Conservative therapy including fasting and transfusion improved the anemia. Echocardiography demonstrated severe aortic stenosis (AS) with a hypertrophied left ventricle. Hematologic examination by gel electrophoresis showed deficiency of high-molecular-weight multimers of von Willebrand factor (vWF), resulting in the diagnosis of Heyde syndrome. He electively underwent aortic valve replacement (AVR) with a bioprosthesis using standard cardiopulmonary bypass. In the perioperative period, the patient had no recurrent anemia. He was discharged without subjective symptoms on postoperative day 18. The 20-month follow-up was unremarkable, with no episode of recurrent GI bleeding. DISCUSSION: Heyde syndrome is associated with acquired von Willebrand disease type IIA and AS. The true characteristic of von Willebrand disease type IIA is hemorrhagic diathesis caused by deficiency of high-molecular-weight multimers of vWF. Under the influence of high shear stress caused by AS, vWF is stretched and easily cleaved by vWF-cleaving protease. Consequently, it causes deficiency of high-molecular-weight multimers of vWF and primary hemostasis impairment. Therefore, the most effective treatment for Heyde syndrome is correction of AS. AVR can improve not only the hemodynamic status but also coagulopathy. CONCLUSION: The differential diagnosis of patients with recurrent GI bleeding with concurrent systolic murmur should include Heyde syndrome. SN - 2210-2612 UR - https://www.unboundmedicine.com/medline/citation/30448636/A_surgical_case_of_aortic_stenosis_with_recurrent_gastrointestinal_bleeding:_Heyde_syndrome_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S2210-2612(18)30502-9 DB - PRIME DP - Unbound Medicine ER -