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Heyde described aortic stenosis and gastrointestinal bleeding in the 1950s. Since then, a link with intestinal angiodysplasia and abnormalities of von Willebrand factor (VWF) has been noted. Loss of the highest-molecular-weight multimers of VWF and bleeding also have been described in subaortic stenosis in hypertrophic cardiomyopathy, in isolated mitral and aortic insufficiency, in endocarditis, in patients with prosthetic valve stenosis or regurgitation, and in patients with left ventricular assist devices (LVADs). Bleeding tends to recur with local treatment of angiodysplasias, whereas cardiac repair or removal of LVAD eliminates VWF dysfunction is curative of bleeding in the majority.

Heyde’s syndrome is defined as the coexistence of aortic valve stenosis (AS) and recurrent gastrointestinal (GI) bleeding from intestinal angiodysplasia (IA). Despite the fact that Heyde’s syndrome was first described decades ago, the association between AS and IA remains frequently unidentified, and thus, a high clinical suspicion is needed for its diagnosis. Here we present a case of a 60-year-old man with a history of severe AS, who presented with recurrent GI bleeding and was found to have multiple intestinal angioectasias on endoscopy.

Gastrointestinal angiodysplasia (GIA) is the most common cause of occult gastrointestinal bleeding (GIB) requiring often hospitalization and transfusions, especially in patients with hemorrhagic disorders. Thalidomide, impairing neo-angiogenesis, has been successfully used in the management of bleeding in patients with GIA and in particular in patients with inherited bleeding disorders. Only one case of short-term treatment with thalidomide in a patient with Glanzmann thrombasthenia (GT) and recurrent GIB due to GIA has been reported so far.We report the case of a woman with GT developing high frequency recurrent GIB due to GIA requiring repeated blood and platelet transfusions, who was treated with thalidomide obtaining a striking and stable reduction of GIB and of the requirement of platelet and blood transfusions for over 5 years. Moreover, we raise the suspicion that the association between GT and GIA may not be fortuitous.

Das Auftreten einer invalidisierenden Osteomalazie bei einem Mann, der wegen intestinalen Angiodysplasien seit Jahren mit intravenosem Eisen behandelt wird, hat an die Rolle des Wachstumsfaktors FGF23 erinnert. FGF23 hemmt, zusammen mit PTH, die renale Phosphat-Ruckresorption und fuhrt zu Hypophosphatamie und Osteomalazie, auch weil es zudem die 25OH Vit.D 1-alpha Hydroxylase hemmt, was wiederum zu einem Mangel an 1,25(OH)2-Vitamin D fuhrt. Die Behandlung besteht in Phosphaten und Calcitriol. Mehrere Medikamente und Krankheiten konnen FGF23 erhohen: mesenchymale Tumoren, multiples Myelom, M. Hodgkin, antivirale Therapien, hypophosphatamische Osteomalazie, Nierentransplantation, intensive Wiederernahrung u. a. So selten die Erhohung des FGF23 als wesentliche Krankheitsursache auch ist, so leicht ist sie zu diagnostizieren. The appearance of a disabling osteomalacia in a man who is treated since many years with intravenous Iron for intestinal angiodysplasia reminded the role of the growth factor FGF23. FGF23, together with PTH, inhibits renal phosphate reabsorption, and causes hypophosphatemia and by that osteomalacia, also because it inhibits the 25OH vit.D 1-alpha hydroxylase, which leads to a deficiency of 1,25(OH)2-vitamin D. The treatment consists in phosphate and Calcitriol. Several drugs and diseases can increase FGF23: mesenchyme tumours, multiple myeloma, M. Hodgkin, antiviral drugs, hypophosphatemic rickets, kidney transplantation, refeeding, etc. As rare the increase of FGF23 appears, as easy it is to recognize it.

Heyde’s syndrome is a form of acquired von Willebrand syndrome that consists of bleeding from intestinal angiodysplasia in the presence of aortic stenosis (AS). An association with obstructive hypertrophic cardiomyopathy, rather than AS, by a similar mechanism has also been described. We report the case of a 78-year-old woman with chronic anaemia and hypertrophic obstructive cardiomyopathy in whom intestinal angiodysplasia with active bleeding was identified by an unconventional method. In this case, the authors describe a different approach to reach the correct diagnosis. LEARNING POINTS In patients with anaemia due to gastrointestinal bleeding, a high level of suspicion is crucial to identify the haemorrhagic focus. Intestinal angiodysplasia is associated with acquired von Willebrand syndrome. Acquired von Willebrand syndrome secondary to hypertrophic obstructive cardiomyopathy occurs by the same mechanism of aortic stenosis.

Heyde's syndrome describes an association between aortic stenosis and a predisposition to bleeding from intestinal angiodysplasia resulting from acquired von Willebrand disease. We present the case of an 82-year-old woman with recurrent intestinal bleeding, severe anaemia and secondary myocardial infarction. Investigation identified ileal angiectasia as the source of haemorrhage. As echocardiography revealed severe aortic stenosis the patient underwent surgical valve replacement. At her 3-month follow-up, the patient reported no new bleeding episodes and her functional status had improved. This case highlights Heyde's syndrome, an entity probably underdiagnosed despite the high prevalence of aortic stenosis and intestinal angiodysplasia in elderly patients. LEARNING POINTS: In a patient with bleeding intestinal angiectasia, Heyde's syndrome should be considered in the differential diagnosis.Although supportive therapy is crucial for clinical stabilization, aortic valve replacement is the therapeutic gold standard.Appropriate management of these patients requires a multidisciplinary approach. info:eu-repo/semantics/publishedVersion

Summary Background To date, there are no large studies on videocapsule endoscopy in systemic sclerosis (SSc). Consequently, the prevalence and features of gastrointestinal mucosal abnormalities in SSc have not been determined. Aims To determine both prevalence and characteristics of gastrointestinal mucosal abnormalities in unselected patients with SSc, using videocapsule endoscopy. To predict which SSc patients are at risk of developing potentially bleeding gastrointestinal vascular mucosal abnormalities. Methods Videocapsule endoscopy was performed on 50 patients with SSc. Results Prevalence of gastrointestinal mucosal abnormalities was 52%. Potentially bleeding vascular mucosal lesions were predominant, including: watermelon stomach (34.6%), gastric and/or small intestinal telangiectasia (26.9%) and gastric and/or small intestinal angiodysplasia (38.5%). SSc patients with gastrointestinal vascular mucosal lesions more often exhibited: limited cutaneous SSc (P = 0.06), digital ulcers (P = 0.05), higher score of nailfold videocapillaroscopy (P = 0.0009), anaemia (P = 0.02), lower levels of ferritin (P

Background: Angiodysplasia of the gastrointestinal tract is an uncommon, but not rare, cause of bleeding and severe anemia in elderly. Different treatments exist for this kind of pathology. Methods: The aim of this work was to study 40 patients treated for intestinal angiodysplasia with two different kind of endoscopic treatments: argon plasma coagulation (APC) and bipolar electrocoagulation (BEC). Results: Age of patients was similar in both groups (76,2 ± 10.8 years vs 74,8 ± 8,7 years, P = 0,005). Angiodysplasia treated were located in small bowel, right colon, left colon, transverse colon and cecum. We analysed number of treatment, recurrence, hospital discharge, needs of blood transfusions before and after endoscopic treatment. Number of treatment was the same in both groups (1,2 ± 0,2 vs 1,1 ± 0,1, P < 0,001). We had more recurrence in patients treated with BEC (4/20 vs 2/20, P < 0,001). Hospital discharge was comparable in both groups (5,3 ± 3,1 days vs 5,4 ± 2,8 years, P < 0.001) Conclusions: Treatment of angiodysplasia in elderly is not easy. Different kinds of treatment could be adopted. APC and BEC are both safe and effective. The choice of a treatment should consider several factors: age, comorbidity, source of bleeding. In conclusion we think that treatment of bleeding for angiodysplasia in elder population should be a tailored treatment.

INTRODUCTION: Angiodysplasia is a rare lesion (0.82% prevalence) characterised by enlarged, abnormally dilated blood vessels in the mucous or submucous sheath of the gastrointestinal tract. This condition clinically manifests with acute or chronic bleeding from the gastrointestinal tract. Typically the diagnosis is based on endoscopic findings and histological characteristics of the distorted vascular structures. Angiodysplasia is quite a common finding in elderly patients. However, the reasons for the anomaly observed with advancing age are yet to be revealed. CASE REPORT: The aim of this report is to highlight three cases of intestinal angiodysplasia histologically confirmed in patients at 47, 60 and 66 years of age. Histological examination using light microscopy revealed different in size and shape dilated, filled with blood vessels surrounded by oedematous, inflamed stroma in the mucosal and submucosal layer of the intestine. CONCLUSION: Angiodysplasia is considered to be the second most common cause of gastrointestinal bleeding. This makes an adequate histologically confirmed diagnosis of this anomaly crucial for a patient`s quality of life.

Heyde’s syndrome is defined as a combination of gastrointestinal bleeding from a gastrointestinal angiodysplasia in patients with aortic stenosis. An acquired von Willebrand factor deficiency secondary to aortic stenosis is considered to be the major abnormality in Heyde’s syndrome. Management of Heyde’s syndrome requires cooperation of many different kinds of specialists: cardiologists, gastroenterologists, hematologists, general surgeons and cardiosurgeon. We present a care report of 62 years old female with no history of cardiovascular disorders referred to the Centre of Rare Cardiovascular Diseases in Krakow for cardiological evaluation of a heart murmur prior to a surgical procedure of a vascular malformation in colon. JRCD 2014; 1 (7): 17–20

Angiodysplasia is one cause of chronic gastrointestinal tract bleeding that can not be difficult to detect. In particular, angiodysplasia in the small intestine is very rare and has seldom been reported. An 80-year-old man complained of hematochezia and was admitted to other hospital on October 1991. However, no bleeding sites in the gastrointestinal tract could be revealed by radiographic and endoscopic examinations. After 8 months, he was admitted to our hospital with the same symptom on June 1992. He suffered hemorrhagic shock due to continuous bleeding. Hypotension and anemia progressed in spite of immediate blood transfusion. Emergency angiography revealed extravasation from a peripheral branch of the jejunal artery, in addition tortuous and dilated ileal arteries. Embolization was performed with the catheter inserted superselectively into the nearest arcade of the segmental branch of the vasa recta. After embolization, no complications were occurred. He was discharged and is being followed up as an outpatient.

We hypothesize the existence of an age-associated neovasculopathy with recurrent bleeding. It could be the result of age-related defects in the regulation of the angiogenetic process creating pathologic small vessels with a bleeding tendency. Conditions like subdural hematoma, ocular angiopathies, intestinal angiodysplasia, coronary atherosclerotic plaques and others, may be the clinical expression of such a neovasculopathy.

Background: This study assessed the long-term effectiveness of push enteroscopic cauterization of bleeding intestinal angiodysplasia. Methods: We retrospectively reviewed the clinical course of patients who underwent push and sonde enteroscopy for obscure gastrointestinal bleeding and were diagnosed with intestinal angiodysplasias. Results: One hundred twelve patients bleeding from small intestinal angiodysplasias were identified. After excluding those lost to follow-up (29), data were collected from 83 patients. Fifty-five patients (29 men; mean age, 73 years; mean units of packed red blood cells transfused, 21.4; average bleeding history, 22 months) were cauterized. Twenty-eight patients (12 men; mean age, 71; mean units of packed red blood cells transfused, 15.8; average bleeding history, 22 months) were not cauterized. The noncauterized group (follow-up, 26 ± 14 months; mean ± SD) continued to bleed, requiring 2.16 ± 3.86 units of packed red blood cells transfused per month (units/month) before and 0.97 ± 1.46 units/month after diagnosis (NS) . The cauterized group (follow-up, 30 ± 18 months) significantly improved, requiring 2.40 ± 2.97 units/month before treatment and 0.32 ± 0.91 units/month after cauterization ( p t test). Conclusion: Cauterization of endoscopically accessible small intestinal angiodysplasias may decrease rebleeding. (Gastrointest Endosc 1996;43:580-3.)