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Revista Española de Enfermedades Digestivas

Print version ISSN 1130-0108

Rev. esp. enferm. dig. vol.104 n.11 Madrid Dec. 2012 



Beyond Heyde's syndrome

Más allá del síndrome de Heyde



Key words: Heyde syndrome. Multiple myeloma. Gastrointestinal bleeding.

Palabras clave: Síndrome de Heyde. Mieloma múltiple. Hemorragia digestiva.



Dear Editor,

The association of aortic stenosis and recurrent gastrointestinal bleeding due to angiodysplasia was first described by Edward G. Heyde in 1958 (1). This so called Heyde syndrome was recently associated with type 2A von Willebrand disease. According to this theory, when von Willerbrand factor pass through the stenotic aortic valve, it is exposed to a high flow, which determined that its multimers are destroyed by a disintegrin (ADAMS 13). Such multimers are necessary to keep hemostasis in high flow conditions such as angiodysplastic arteriovenous malformations, frequently observed in the elderly (2). Aortic valve replacement has been proposed to treat this syndrome when a significant anemia is detected and medical and endoscopic treatment failed (3).


Case report

A 62-years-old female patient with a past history of double aortic disease (severe stenosis and moderate insufficiency) with preserved systolic function. The patient was admitted in another hospital two years before due to anemia and melena. Gastroscopy and colonoscopy were normal. Endoscopic capsule was performed and a bleeding spot was detected in the jejunum, followed by enteroscopy with sclerosis and marking of the two vascular lesions of the jejunum. CT scan was normal and tumor markers were negative. One year later, the patient was hospitalized in our unit due to the same symptoms. Gammagraphy with octreotide was normal and an arteriography detected an arterial malformation in the second jejunal branch of the superior mesenteric artery. A new enteroscopy identified a new jejunal lesion distal to the previous ones which was coagulated with APC with successful hemostasis. A Heyde syndrome was suspected after cardiac failure, therefore aortic replacement with a biologic prosthesis was performed. Even though the patient did not have melena, slight leucopenia, trombopenia and anemia was detected in the blood count. A proteinogram was performed identifying slight monoclonal IgG gammapathy and Bence-Jones proteinuria.



The prevalence of Heyde syndrome is unknown. The diagnosis has to be made ruling out other possibilities and requires the triad of aortic stenosis, adquired coagulopaty and anemia due to bleeding secondary to telangiectasias (intestinal or any other location) (4,5). Even though all three conditions were observed and the valve replacement was considered curative, her anemia did not improve. A more detailed study lead us to diagnosis of stage IA multiple myeloma. This study was not initially performed because we considered the anemia was secondary to gastrointestinal bleeding. We report this case considering an infrequent association of these two diseases and particularly because Heyde syndrome did not respond to treatment and enabled the diagnosis of another disease which caused anemia.


Alejandro Martínez-Caselles, Cristina Martínez-Pascual, Antonio Sánchez-Torres and Fernando Carballo-Álvarez
Department of Digestive Diseases. Hospital Universitario Virgen de la Arrixaca. Murcia, Spain



1. Vaz A, Correia A, Martins B, Capelo J, Fencina P, Henriques P, et al. Heyde syndrome-The link between aortic stenosis and gastrointestinal bleeding. Rev Port Cardiol 2010;29:309-14.         [ Links ]

2. Massyn MW, Khan SA. Heyde syndrome: a common diagnosis in older patients with severe aortic stenosis. Age and Ageing 2009;38:267-70.         [ Links ]

3. Morishima A, Marui A, Shimamoto T, Saji Y, Tambara K, Nishina T, et al. Successful aortic valve replacement for Heyde syndrome with confirmed hematologic recovery. Ann Thorac Surg 2007;89:287-8.         [ Links ]

4. Giovannini I, Chiarla C, Murazio M, Clemente G, Giulante F, Nuzzo G. An extreme case of Heyde syndrome. Dig Surg 2006;23:387-8.         [ Links ]

5. Henne S, Denzer U, Seitz U, Göttsche J, Soehendra N, Lohse A. Rezidivierende gastrointestinale Blutungen bei Aortenklappenstenose (Heyde-Syndrom): Indikation zum Aortenklappenersatz? Z Gastroenterol 2007;45:245-9.         [ Links ]

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