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Gastrointestinal Bleeding due to Angiodysplasia in a Patient with Severe Aortic Stenosis: Heyde’s Syndrome
Felipe Homem Valle1,
Autor para correspondencia
felipehvalle@gmail.com

Felipe Homem Valle. Serviço de Cardiologia do Hospital de Clínicas de Porto Alegre. R. Ramiro Barcelos, 2.350 – Bairro Rio Branco – Porto Alegre, RS, Brazil – CEP 90035-903
, Fernando Pivatto Júnior2, Luiz Carlos Corsetti Bergoli3, Rodrigo Vugman Wainstein4, Marco Vugman Wainstein5
1 Resident physician at the Cardiology Service of Hospital de Clínicas de Porto Alegre. Porto Alegre, RS, Brazil.
2 Resident physician at the Cardiology Service of Hospital de Clínicas de Porto Alegre. Porto Alegre, RS, Brazil.
3 Hired physician at the Cardiology Service of Hospital de Clínicas de Porto Alegre – Unidade de Hemodinâmica. Porto Alegre, RS, Brazil.
4 Hired physician at the Cardiology Service of Hospital de Clínicas de Porto Alegre – Unidade de Hemodinâmica. Porto Alegre, RS, Brazil.
5 Adjunct professor of Faculdade de Medicina da Universidade Federal do Rio Grande do Sul. Porto Alegre, RS, Brazil.
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="p0005" class="elsevierStylePara elsevierViewall">Heyde&#8217;ssyndrome&#44; an association between aortic stenosis and gastrointestinal bleeding&#44; was first described by Edward Heyde in 1958&#44;<a class="elsevierStyleCrossRef" href="#bb0005"><span class="elsevierStyleSup">1</span></a> in a series of ten patients with aortic stenosis and gastrointestinal bleeding of unknown cause&#44; and the association with intestinal angiodysplasia was only established subsequently&#46; Gastrointestinal bleeding appears to be caused by acquired von Willebrand disease type 2A&#44; characterized by a reduction in high molecular weight multimers of von Willebrand factor &#40;VWF&#41;&#46; These multimers are important to maintain hemostasis in situations of high shear stress &#8211; predominant flow conditions in patients with angiodysplastic lesions&#46;<a class="elsevierStyleCrossRef" href="#bb0010"><span class="elsevierStyleSup">2</span></a> Valve replacement appears to offer the best hope for a long-term resolution of bleeding&#44; and should beconsidered in most cases&#46;&#160;<a class="elsevierStyleCrossRef" href="#bb0015"><span class="elsevierStyleSup">3</span></a> The case of a patient with severe aortic stenosis and gastrointestinal bleeding due to duodenal angiodysplasia is reported&#44; successfully treated by transcatheter aortic valve implantation&#46;</p><span id="s0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="st0025">CASE REPORT</span><p id="p0010" class="elsevierStylePara elsevierViewall">Female patient&#44; 79 years&#44; with severe aortic stenosis &#40;valve area of 0&#46;6<span class="elsevierStyleHsp" style=""></span>cm<a class="elsevierStyleCrossRef" href="#bb0010"><span class="elsevierStyleSup">2</span></a>&#59; maximum transvalvular gradient of 96<span class="elsevierStyleHsp" style=""></span>mmHg and mean of 59<span class="elsevierStyleHsp" style=""></span>mmHg&#59; and preserved left ventricular systolic function&#41; was hospitalized for symptomatic heart failure associated with melena&#46; On admission&#44; hemoglobin level was 4&#46;6<span class="elsevierStyleHsp" style=""></span>g&#47;dL&#46; She was initially submitted to endoscopic gastrointestinal tract assessment&#44; which showed no alterations&#44; and colonoscopy&#44; which showed diverticular disease of the colon&#44; with no evidence of recent bleeding&#46; After receiving transfusion of packed red blood cells&#44; she achieved partial symptom improvement&#46;</p><p id="p0015" class="elsevierStylePara elsevierViewall">After the case study&#44; it was decided to perform the transcatheter aortic stenosis treatment&#46; A 23-mm Edwards SAPIEN XT valve &#40;Edwards Life Sciences &#8211; Irvine&#44; USA&#41; was implanted by transfemoral route&#46; The procedure was successfully performed without complications&#44; resulting in aortic transvalvular gradient of 10<span class="elsevierStyleHsp" style=""></span>mmHg with no significant aortic regurgitation &#40;<a class="elsevierStyleCrossRef" href="#f0005">Figure&#160;1</a>&#41;&#46; Two days after the procedure&#44; there was recurrence of melena&#46; At that time&#44; it was decided to evaluate the small bowel by enteroscopy&#44; for clarification of the bleeding site&#46; The enteroscopy evaluated the bowel up to the medium jejunum&#44; showing angiodysplastic lesion in the proximal duodenum &#40;<a class="elsevierStyleCrossRef" href="#f0010">Figure&#160;2</a>&#41;&#44; which was cauterized with an argon plasma catheter&#46; The patient was discharged to be followed by her cardiologist in good clinical conditions&#44; with hemoglobin of 8&#46;2<span class="elsevierStyleHsp" style=""></span>g&#47;dL&#44; 21 days after admission&#46; She was using acetylsalicylic acid &#40;ASA&#41; 100<span class="elsevierStyleHsp" style=""></span>mg&#47;day and clopidogrel 75<span class="elsevierStyleHsp" style=""></span>mg&#47;day was prescribed &#40;dual antiplatelet therapy&#41;&#44; after the absence of intestinal bleeding recurrence was confirmed&#44; seven days after discharge&#46;</p><elsevierMultimedia ident="f0005"></elsevierMultimedia><elsevierMultimedia ident="f0010"></elsevierMultimedia></span><span id="s0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="st0030">DISCUSSION</span><p id="p0020" class="elsevierStylePara elsevierViewall">Intestinal angiodysplasia and aortic stenosis are usually asymptomatic chronic&#8211;degenerative diseases&#44; whose prevalence increases with age&#46; Both are associated with cardiovascular risk factors&#46; Many studies suggest that there is a greater occurrence of intestinal stenosis in subjects with aortic angiodysplasia&#44; and vice versa&#46;<a class="elsevierStyleCrossRef" href="#bb0020"><span class="elsevierStyleSup">4</span></a> The main concept behind this association is based on the fact that both angiodysplasia intestinal and aortic stenosis are related to the physiological processes of aging&#46;<a class="elsevierStyleCrossRef" href="#bb0025"><span class="elsevierStyleSup">5</span></a></p><p id="p0025" class="elsevierStylePara elsevierViewall">In 1992&#44; an etiopathogenic mechanism was proposed by Warkentin et al&#46;&#44;<a class="elsevierStyleCrossRef" href="#bb0010"><span class="elsevierStyleSup">2</span></a> according to which the acquired deficiency of high molecular weight multimers of VWF &#40;von Willebrand disease type 2A&#41;&#44; necessary to maintain hemostasis in high-flow conditions&#44;<a class="elsevierStyleCrossRef" href="#bb0020"><span class="elsevierStyleSup">4</span></a> as occurs in arteriovenous angiodysplastic malformations&#44; appeared to be the connection between the two components of this syndrome&#46; The VWF is secreted by endothelial cells into the blood&#44; contributing to platelet thrombus formation and acting as a mediator of platelet adhesion at the site of vascular injury&#46;<a class="elsevierStyleCrossRef" href="#bb0030"><span class="elsevierStyleSup">6</span></a> The high shear stress caused by the stenotic valve results in changes in the VWF<a class="elsevierStyleCrossRef" href="#bb0025"><span class="elsevierStyleSup">5</span></a> molecule structure and subsequent proteolysis of the high molecular weight multimers by ADAMTS13 enzyme&#46; The multimers with reduced size are&#44; from the hemostatic point of view&#44; less competent than the intact large multimers&#46; This hypothesis is supported by the fact that the change of the stenotic aortic valve results in long&#8211;term resolution of digestive bleeding&#46; In summary&#44; the development of the acquired von Willebrand disease type 2A due to aortic stenosis causes hemostatic abnormalities&#44; which predispose to intestinal angiodysplastic bleeding&#44; previously subclinical&#46;<a class="elsevierStyleCrossRef" href="#bb0025"><span class="elsevierStyleSup">5</span></a></p><p id="p0030" class="elsevierStylePara elsevierViewall">A study by Vincentelli et al&#46;<a class="elsevierStyleCrossRef" href="#bb0035"><span class="elsevierStyleSup">7</span></a> demonstrated that the degree of aortic stenosis is proportional to hemostatic changes&#58; the higher the gradient pressure&#44; the lower the availability of high molecular weight multimers&#46; In this study&#44; the aortic valve replacement ceased the depletion of these multimers&#44; and this effect has been observed in some patients as soon as the first postoperative day&#46;<a class="elsevierStyleCrossRef" href="#bb0025"><span class="elsevierStyleSup">5</span></a></p><p id="p0035" class="elsevierStylePara elsevierViewall">Regarding gastrointestinal bleeding due to intestinal angiodysplasia&#44; the options for treatment include the location and cauterization of lesions&#59; however&#44; these strategies are associated with high rate of recurrence&#46; The improvement in hematological alterations after aortic valve replacement has been described and is also accompanied by clinical improvement&#58; this approach seems to offer the best prospects for long-term resolution of bleeding and should be considered in most cases&#44; especially those with symptomatic aortic stenosis&#46; For patients at high surgical risk&#44; in which no bleeding point was identified&#44; the treatment of anemia with regular blood transfusions can provide some symptomatic relief&#46;<a class="elsevierStyleCrossRef" href="#bb0020"><span class="elsevierStyleSup">4</span></a></p><p id="p0040" class="elsevierStylePara elsevierViewall">Aortic valve replacement appears to reduce the risk of gastrointestinal hemorrhage in patients with Heyde&#8217;s syndrome&#44; resolving the condition in approximately 80&#37; of cases&#46; Although the rates of bleeding recurrence are not significantly different between biological and mechanical valves&#44; the increased risk of bleeding in patients receiving anticoagulants makes biological valves the choice for most patients&#46;<a class="elsevierStyleCrossRef" href="#bb0040"><span class="elsevierStyleSup">8</span></a> Bleeding recurrence occurs only when the physiopathological mechanism is restored&#44; either by aortic restenosis or by patientprosthesis mismatch &#40;effective valvearea&#47;bodysurface &#60; 0&#46;85<span class="elsevierStyleHsp" style=""></span>cm<span class="elsevierStyleSup">2</span>&#47;m<span class="elsevierStyleSup">2</span> on echocardiography&#41;&#44; with elevated gradients persisting postoperatively even with normal functioning valves&#46;<a class="elsevierStyleCrossRef" href="#bb0035"><span class="elsevierStyleSup">7</span></a></p><p id="p0045" class="elsevierStylePara elsevierViewall">This case report is the second article that addresses the percutaneous treatment of aortic stenosis in patients with Heyde&#8217;s syndrome&#46;<a class="elsevierStyleCrossRef" href="#bb0045"><span class="elsevierStyleSup">9</span></a> Godino et al&#46;<a class="elsevierStyleCrossRef" href="#bb0045"><span class="elsevierStyleSup">9</span></a> reported that this syndrome was present in 1&#46;7&#37; of 400 patients undergoing transcatheter aortic valve implantationat their institution&#44; and that the recurrence of gastrointestinal bleeding was abolished in all successfully treated patients&#46;</p></span><span id="s0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="st0035">CONFLICTS OF INTEREST</span><p id="p0050" class="elsevierStylePara elsevierViewall">Marco Vugman Wainsteinis a consultant for Biotronik&#44; B Braun&#44; and Terumo&#59; board member of A esculap B Braun&#59; researcher for Biotronik&#59; and lector for Biosensors&#44; B Braun&#44; Terumo&#44; and Astra Zeneca&#59; he also receives scientific support from Abbott Vascular&#44; Cordis J&#38;J&#44; and Boston Scientific&#46; The remaining authors declare to have no conflicts of interests&#46;</p></span></span>"
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        "resumen" => "<span id="as0010" class="elsevierStyleSection elsevierViewall"><p id="sp0015" class="elsevierStyleSimplePara elsevierViewall">There is a frequent association between aortic valve stenosis and gastrointestinal bleeding&#44; also known as Heyde&#8217;s syndrome&#46; The pathophysiologic basis for this syndrome seems to be an acquired von Willebrand factor deficiency&#44; leading to bleeding of angiodysplastic arteriovenous malformations&#46; Treatment options include the location and cauterization of bleeding points&#44; which is associated to high recurrence rates&#46; Valve replacement appears to offer the best hope of long-term resolution of bleeding&#44; and should be considered in most cases&#46; We report a 79 year-old patient with aortic stenosis and gastrointestinal bleeding due to duodenal angiodysplasia treated by transcatheter aortic valve implantation&#46;</p></span>"
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        "resumen" => "<span id="as0015" class="elsevierStyleSection elsevierViewall"><p id="sp0020" class="elsevierStyleSimplePara elsevierViewall"><span class="elsevierStyleBold">Sangramento Gastrintestinal por</span><span class="elsevierStyleBold">Angiodisplasia e Estenose A&#243;rtica&#58; S&#237;ndrome de Heyde</span></p><p id="sp0025" class="elsevierStyleSimplePara elsevierViewall">Existe uma associa&#231;&#227;o frequente entre estenose valvar a&#243;rtica e sangramento gastrintestinal&#44; tamb&#233;m conhecida como s&#237;ndrome de Heyde&#46; A base fisiopatol&#243;gica dessa s&#237;ndrome parece ser uma defici&#234;ncia adquirida do fator de von Willebrand&#44; que leva ao sangramento de malforma&#231;&#245;es arteriovenosas angiodispl&#225;sicas&#46; As alternativas de tratamento incluem a localiza&#231;&#227;o de pontos de sangramento e a cauteriza&#231;&#227;o&#44; mas tal op&#231;&#227;o terap&#234;utica est&#225; associada a alta recorr&#234;ncia&#46; A substitui&#231;&#227;o da v&#225;lvula parece oferecer a melhor esperan&#231;a de resolu&#231;&#227;o a longo prazo do sangramento e deve ser considerada na maioria das vezes&#46; Relatamos aqui o caso de uma paciente de 79 anos com estenose a&#243;rtica e sangramento gastrintestinal devido &#224; angiodisplasia duodenal&#44; tratada por implante transcateter de v&#225;lvula a&#243;rtica&#46;</p></span>"
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Información del artículo
ISSN: 22141235
Idioma original: Portugués
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2021 Julio 47 8 55
2021 Junio 45 6 51
2021 Mayo 48 10 58
2021 Abril 81 26 107
2021 Marzo 33 9 42
2021 Febrero 70 9 79
2021 Enero 38 6 44
2020 Diciembre 44 2 46
2020 Noviembre 22 5 27
2020 Octubre 7 3 10
2020 Septiembre 17 8 25
2020 Agosto 8 2 10
2020 Julio 11 3 14
2020 Junio 4 1 5
2020 Mayo 5 2 7
2020 Abril 5 2 7
2020 Marzo 11 0 11
2020 Febrero 10 1 11
2020 Enero 7 3 10
2019 Diciembre 9 3 12
2019 Noviembre 2 2 4
2019 Octubre 4 2 6
2019 Septiembre 4 2 6
2019 Agosto 2 0 2
2019 Julio 3 7 10
2019 Junio 13 11 24
2019 Mayo 14 4 18
2018 Octubre 1 0 1
2018 Septiembre 5 1 6
2018 Agosto 28 14 42
2018 Julio 21 10 31
2018 Junio 27 12 39
2018 Mayo 30 10 40
2018 Abril 32 18 50
2018 Marzo 45 23 68
2018 Febrero 34 14 48
2018 Enero 17 6 23
2017 Diciembre 28 9 37
2017 Noviembre 15 7 22
2017 Octubre 16 10 26
2017 Septiembre 21 11 32
2017 Agosto 18 7 25
2017 Julio 20 12 32
2017 Junio 24 18 42
2017 Mayo 58 24 82
2017 Abril 277 131 408
2017 Marzo 23 12 35
2017 Febrero 20 9 29
2017 Enero 30 12 42
2016 Diciembre 38 12 50
2016 Noviembre 27 7 34
2016 Octubre 24 7 31
2016 Septiembre 16 4 20
2016 Agosto 13 6 19
2016 Julio 7 6 13
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