Heyde’s syndrome

Česká verze

Authors: Michalová R. ¹; Šimonová R. ²; Michalová R. jr. ³; P. Bánovčin Jr. ¹ ; Hyrdel R. ¹
Authors place of work: Interná klinika gastroenterologická JLF UK a UN Martin, 2 Klinika hematológie a transfuziológie JLF UK a UN Martin, 3 I. interná klinika JLF UK a UN Martin ¹
Published in the journal: Gastroent Hepatol 2021; 75(4): 298-303
Category: Klinická a experimentální gastroenterologie: přehledová práce
doi: https://doi.org/10.48095/ccgh2021298

Summary

Gastrointestinal bleeding is a severe and potentially life-threatening condition associated with significant morbidity and mortality. In clinical practice, its differential diagnosis can sometimes be complicated, especially when bleeding is recurrent. One of the lesser-known reasons for recurrent gastrointestinal bleeding is the so called Heyde’s syndrome. It is a combination of bleeding from gastrointestinal angiodysplasia, aortic stenosis and acquired type 2A von Willebrand syndrome. In the submitted article, authors present a summary of the current knowledge on etiology and pathogenesis of the disease. The degenerative aortic valve disease, typically present in elderly patients, causes changes in the blood flow through stenotic opening. This results in elongation of otherwise globular von Willebrand’s factor (vWF) molecules, resulting in its reduced efficacy in hemostasis. According to new scientific discoveries, vWF also plays a role in angiogenesis and therefore in gastrointestinal angiodysplasia formation. This combination of factors results in recurrent bleeding typical for Heyde’s syndrome. Surgical aortic valve replacement is the gold standard treatment. A vicious circle can often be created between the gastroenterologist, who refers the patient for cardiac surgery procedures, and the cardiothoracic surgeon, who is waiting for a time when the patient will stop bleeding. A close communication and cooperation between the gastroenterologist, hematologist and cardiothoracic surgeon is required to properly manage the patient. The presence of angiodysplasia in an elderly patient, associated with recurrent gastrointestinal bleeding, should lead clinicians to look for aortic stenosis and consider Heyde’s syndrome as a potential diagnosis. Its interesting etiopathogenesis and rare occurrence led the authors to further investigate this topic. They also present a short summary of their own group of patients.

Keywords:

Heyde’s syndrome – aortic stenosis – angiodysplasia – von Willebrand’s syndrome

Zdroje

1. Heyde EC. Gastrointestinal bleeding in aortic stenosis. N Engl J Med 1958; 259: 196.

2. Greenstein RJ, McElhinney AJ, Reuben D et al. Colonic vascular ectasias and aortic stenosis: coincidence or causal relationship? Am J Surg 1986; 151 (3): 347–351. doi: 10.1016/0002-9610 (86) 90465-4.

3. Warkentin TE, Moore JC, Morgan DG. Aortic stenosis and bleeding gastrointestinal angiodysplasia: is acquired von Willebrand‘s disease the link? Lancet 1992; 340 (8810): 35–37. doi: 10.1016/0140-6736 (92) 92434-h.

4. Sadler JE. Aortic stenosis, von Willebrand factor, and bleeding. N Engl J Med 2003; 349 (4): 323–325. doi: 10.1056/NEJMp030055.

5. Hudzik B, Wilczek K, Gasior M. Heyde syndrome: gastrointestinal bleeding and aortic stenosis. CMAJ 2016; 188 (2): 135–138. doi: 10.1503/cmaj.150194.

6. Islam S, Cevik C, Islam E et al. Heyde‘s syndrome: a critical review of the literature. J Heart Valve Dis 2011; 20 (4): 366–375.

7. Sami SS, Al-Araji SA, Ragunath K. Review article: gastrointestinal angiodysplasia – pathogenesis, diagnosis and management. Aliment Pharmacol Ther 2014; 39 (1): 15–34. doi: 10.1111/apt.12527.

8. Regula J, Wronska E, Pachlewski J. Vascular lesions of the gastrointestinal tract. Best Pract Res Clin Gastroenterol 2008; 22 (2): 313–328. doi: 10.1016/j.bpg.2007.10.026.

9. Chukwudum CA, Vera S, Sharma M et al. Heyde syndrome: a case report and literature review. Cureus 2020; 12 (4): e7896. doi: 10.7759/cureus.7896.

10. Pate GE, Chandavimol M, Naiman SC et al. Heyde‘s syndrome: a review. J Heart Valve Dis 2004; 13 (5): 701–712.

11. Šimonová R, Sokol J, Chudej J et al. Heydeho syndróm: etiopatogenéza, diagnostia a liečba. Vask Med 2014; 6 (2): 70–72.

12. Gola W, Lelonek M. Clinical implication of gastrointestinal bleeding in degenerative aortic stenosis: an update. Cardiol J 2010; 17 (4): 330–334.

13. Franchini M, Mannucci PM. Von Willebrand disease-associated angiodysplasia: a few answers, still many questions. Br J Haematol 2013; 161 (2): 177–182. doi: 10.1111/bjh.12272.

14. Starke RD, Ferraro F, Paschalaki KE et al. Endothelial von Willebrand factor regulates angiogenesis. Blood 2011; 117 (3): 1071–1080. doi: 10.1182/blood-2010-01-264507.

15. Škorňová I, Staško J, Hollý P et al. Von Willebrandova choroba – porovanie dvoch metód analýzy multimérov von Willebrandovho faktora. Transfuze Hematol Dnes 2021; 27 (1): 42–50. doi: 10.48095/cctahd202142.

16. Akutagawa T, Shindo T, Yamanouchi K et al. Persistent Gastrointestinal Angiodysplasia in Heyde‘s Syndrome after Aortic Valve Replacement. Intern Med 2017 15; 56 (18): 2431–2433. doi: 10.2169/internalmedicine.8603-16.

17. Loscalzo J. From clinical observation to mechanism – Heyde‘s syndrome. N Engl J Med 2012; 367 (20): 1954–1956. doi: 10.1056/NEJMcibr 1205363.

18. Theis SR, Turner SD. Heyde syndrome. Treasure Island (FL): StatPearls Publishing 2021.

19. Thompson JL 3rd, Schaff HV, Dearani JA. Risk of recurrent gastrointestinal bleeding after aortic valve replacement in patients with Heyde syndrome. J Thorac Cardiovasc Surg 2012; 144 (1): 112–116. doi: 10.1016/j.jtcvs.2011.05. 034.