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Table of Contents
LETTER TO THE EDITOR
Year : 2012  |  Volume : 6  |  Issue : 1  |  Page : 54-55

Heyde's syndrome: An unusual cause of lower gastrointestinal bleeding


Department of Gastroenterology, Dr. Ram Manohar Lohia Hospital and PGIMER, New Delhi, India

Date of Web Publication28-Aug-2012

Correspondence Address:
Nandan Mishra
Department of Gastroenterology, Dr. Ram Manohar Lohia Hospital and PGIMER, New Delhi-110001
India
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DOI: 10.4103/0331-3131.100230

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How to cite this article:
Mishra N, Moda P, Khanna A. Heyde's syndrome: An unusual cause of lower gastrointestinal bleeding. Ann Nigerian Med 2012;6:54-5

How to cite this URL:
Mishra N, Moda P, Khanna A. Heyde's syndrome: An unusual cause of lower gastrointestinal bleeding. Ann Nigerian Med [serial online] 2012 [cited 2014 Nov 27];6:54-5. Available from: http://www.anmjournal.com/text.asp?2012/6/1/54/100230

Sir,

Lower gastrointestinal bleed is a common medical-surgical emergency. A rather uncommon cause of it is Heyde's syndrome, which is the combination of calcific aortic stenosis and gastrointestinal bleeding due to angiodysplasia. [1] A patient presented with lower gastrointestinal bleed and was initially managed in a routine manner until we stumbled upon the valvular aortic stenosis. This case forces us to discuss the relationship between these two clinical entities and highlights the importance of a thorough clinical examination in this context.

A 79-year-old male presented with generalized weakness, breathlessness on exertion, and passage of fresh blood with defecation, on and off for 1 year for which he had been on diuretics and hematinic. The patient had been treated for hypertension about 10 years previously, but was presently not on any medication. When he presented to us, he had been having hematochezia over the preceding 2 days. On physical examination his vitals were stable, blood pressure was 130/90 mm Hg, and apart from the presence of severe pallor, there were no discernible clinical findings. Investigations revealed hypochromic microcytic anemia, serum iron studies suggested severe iron-deficiency anemia. Coagulation profiles, chest X-ray, and abdominal ultrasound scan were normal. The stool sample had visible streaking with blood which was confirmed by testing of the stool sample.

ECG was suggestive of LVH (Sokolow--Lyon index positive), which was thought to be compatible with the past history of hypertension. Digital rectal examination and a preliminary sigmoidoscopy after an urgent oral purge with polyethylene glycol solution were normal. The patient was then resuscitated while a proper colonoscopy was being planned. He was again examined by the resident on duty, who could appreciate a soft ejection systolic murmur in the right parasternal area, with selective radiation to the neck; this had been missed in the earlier examination. An urgent screening transthoracic echocardiography was performed.

The 2D-Echo was suggestive of situs solitus, normal concordance, with a mean gradient of 67 mm Hg across the aortic valve with a valve area of 0.6 cm 2 , trivial mitral and tricuspid regurgitation were reported. Ejection fraction was 62%. The overall picture suggested severe aortic stenosis.

The upper gastrointestinal endoscopy was normal. The colonoscopy revealed a 5 mm × 5 mm coral reef-shaped vascular mass suggestive of angiodysplasia in the proximal ascending colon, just distal to the terminal cecum. The lesion was not actively bleeding at the time of colonoscopy; neither could we appreciate a clot surrounding the lesion.

Keeping the possibility of Heyde's syndrome in mind, selective mesenteric angiography which is recommended as a "gold standard" [2] was done which showed a typical angiodysplastic lesion with early and late feeder vessels and electrophoretic analysis of Von-Willebrand factor multimers showed that the larger multimers were decreased. These findings are commensurate with the diagnosis of Heyde's syndrome.

Preoperative workup for aortic valve replacement surgery was planned for the patient.but unfortunately, the patient and his attendants refused any surgery or colonoscopic procedure after this and left the hospital against medical advice.

Intestinal angiodysplasia and valvular aortic stenosis are both chronic degenerative diseases that are often asymptomatic. The incidence of both conditions increases with age and both are associated with traditional cardiovascular risk factors. According to the different studies conducted to evaluate the association between aortic stenosis and gastrointestinal dysplasia, about 7-29% [3],[4] of the patients with diagnosed gastrointestinal angiodysplasia suffer from aortic stenosis, and 3% of advanced aortic stenosis patients have gastrointestinal bleeding. [3]

In these patients, it is postulated that the bleeding of angiodysplasia is triggered by the uncorrected severe aortic stenosis. When blood passes through the narrowed aortic valve, the turbulent flow generated causes mechanical disruption of the Von-Willebrand's multimers. The deficiency of Von-Willebrand's factor leads to a secondary reduction in plasma factor VIII levels and impaired platelet adherence to the subendothelial components. Because of this acquired hematological defect, also known as Von-Willebrand's syndrome, patients with uncorrected aortic stenosis are more prone to bleed from previously latent angiodysplasia. [5]

Aortic valve replacement is recommended as a "gold standard." [6],[7] It has been reported that 95% of patients with aortic stenosis who have gastrointestinal bleeding due to angiodysplasia have no bleeding after aortic valve replacement. [8]

Other treatment modalities include gastrointestinal surgery such as segmental bowel resection or blind right colectomy. In patients deemed unfit for surgery the alternatives may be selective embolization during mesenteric angiography, localization of angiodysplastic bleeding points endoscopically followed by photocoagulation, electrocoagulation, sclerotherapy or hormonal therapy.

Hematochezia is a common clinical problem faced by patients globally. Unlike in Western countries, it is not easy in our setup to carry out the proper workup of these patients as the facilities for colonoscopy and angiography are not freely available. This report serves to alert physicians to the possible association of severe aortic stenosis with bleeding angiodysplasia. The diagnosis of obscure gastrointestinal bleeding has always been difficult and definitive management is often delayed. Early diagnosis and appropriate treatment of Heyde's syndrome is essential but requires teamwork and liaison between different specialties.

 
   References Top

1.Heyde EC. Gastrointestinal bleeding in aortic stenosis. N Engl J Med 1958;259:196.  Back to cited text no. 1
    
2.Foutch PG. Angiodysplasia of the gastrointestinal tract. Am J Gastroenterol 1993;88:807-18.  Back to cited text no. 2
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3.Mishra PK, Kovac J, Caestecker J, Fancourt G, Logtens E, Spyt T. Intestinal angiodysplasia and aortic valve stenosis: Let's not close the book on this association. Eur J Cardiothorac Surg 2009;35:628-34.  Back to cited text no. 3
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4.Shoenfeld Y, Eldar M, Bedazovsky M. Aortic stenosis associated with gastrointestinal bleeding. A survey of 612 patients. Am Heart J 1980;100:179-82.  Back to cited text no. 4
    
5.Vincentelli A, Susen S, Le Tourneau T. Acquired von Willebrand syndrome in aortic stenosis. N Engl J Med 2003;349:343-9.  Back to cited text no. 5
    
6.Anderson RP, McGrath K, Street A. Reversal of aortic stenosis, bleeding gastrointestinal angiodysplasia, and von Willebrand syndrome by aortic valve replacement. Lancet 1996;347:689-890.  Back to cited text no. 6
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7.Cappell MS, Lebwohl O. Cessation of recurrent bleeding from gastrointestinal angiodysplasias after aortic valve replacement. Ann Intern Med 1986;105:54-7.  Back to cited text no. 7
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8.Love JW. The syndrome of calcific aortic stenosis and gastrointestinal bleeding: Resolution following aortic valve replacement. J Thorac Cardiovasc Surg 1982;83:779-83.  Back to cited text no. 8
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