Aortic Valve Replacement for Patients with Heyde Syndrome: A Literature Review / 中国医学科学杂志(英文版)

Objective To explore the characteristics and clinical outcomes of patients with Heyde syndrome (HS) who undergo aortic valve replacement (AVR). Methods Electronic databases including PubMed, Embase, Ovid, WANFANG, VIP and CNKI were searched to identify all case reports of HS patients undergoing AVR surgery, using different combinations of search terms "Heyde syndrome", "gastrointestinal bleeding", "aortic stenosis", and "surgery". Three authors independently extracted the clinical data including the patients' characteristics, aortic stenosis severity, gastrointestinal bleeding sites, surgical treatments and prognosis. Results Finally, 46 case reports with 55 patients aging from 46 to 87 years, were determined eligible and included. Of them, 1 patient had mild aortic stenosis, 1 had moderate aortic stenosis, 42 had severe aortic stenosis, and 11 were not mentioned. Gastrointestinal bleeding was detected in colon (

El síndrome de Heyde / Heyde syndrome / Síndrome de Heyde

Desde que Edward Heyde vislumbró en 1958 una misteriosa asociación entre estenosis aórtica y hemorragia digestiva han transcurrido seis décadas y se ha suscitado no poca controversia. En la época en que fue propuesta, el estatus técnico y metodológico de la ciencia médica y una interpretación sesgada de su idea original impidieron obtener un sustento estadístico y fisiopatológico que le otorgara un amplio reconocimiento como entidad clínica individual. Los avances en varias disciplinas permitieron demostrar que su frecuencia de presentación excede el efecto del azar, además de esclarecer con precisión y elegancia sus mecanismos fisiopatológicos. Su consolidación como síndrome nos revela una verdadera encrucijada entre la cardiología, la gastroenterología, la hematología y el laboratorio, especialidades involucradas tanto en su proceso de comprensión como en su manejo práctico en la actualidad. Sin embargo, a pesar de tener una incidencia no desdeñable y adquirir un papel central en la conducción clínica de la estenosis aórtica, esta entidad parece haber pasado de ser resistida a relativamente ignorada. Con el objetivo de contribuir a su visibilidad, la presente revisión ofrece un panorama integral sobre el tema, incluyendo una perspectiva histórica de los principales aportes en pos de su conocimiento y un abordaje en profundidad de sus mecanismos, las claves de su detección clínica y su impacto en el manejo de la estenosis aórtica y otras entidades con fisiopatología afín.

Since Edward Heyde perceived in 1958 a mysterious association between aortic stenosis and gastrointestinal bleeding, six decades have passed and no little controversy has arisen. At the time it was proposed, the technical and methodological status of medical science and a biased interpretation of his original idea prevented obtaining a statistical and pathophysiological support that would grant it wide recognition as and individual clinical entity. Advances in several disciplines allowed to demonstrate that its frequency of presentation exceeds the effect of chance, besides clarifying with precision and elegance its pathophysiological mechanisms. Its consolidation as a syndrome reveals a true crossroads between Cardiology, Gastroenterology, Hematology and Laboratory, specialties involved both in its understanding process and in its practical management today. However, despite having a not negligible incidence and acquiring a central role in the clinical conduction of aortic stenosis, this entity seems to have gone from being resisted to relatively ignored. With the objective of contributing to its visibility, this review offers a comprehensive overview of the subject, covering the main historical contributions to its knowledge and approaching in depth its mechanisms, the keys to its clinical detection and its impact on the management of aortic stenosis and other entities with related pathophysiology.

Desde que Edward Heyde imaginou em 1958 uma misteriosa associação entre estenose aórtica e hemorragia gastrointestinal, seis décadas se passaram e não houve pouca controvérsia. Na época em que foi levantada, o estado técnico e metodológico da ciência médica e uma interpretação tendenciosa de sua ideia original impediram a construção de um apoio estatístico e fisiopatológico que lhe concederia amplo reconhecimento como entidade clínica individual. Avanços em diversas disciplinas permitiram demonstrar que sua frequência de apresentação excede o efeito do acaso, além de esclarecer com precisão e elegância seus mecanismos fisiopatológicos. Sua consolidação como síndrome revela uma verdadeira encruzilhada entre Cardiologia, Gastroenterologia, Hematologia e Laboratório, especialidades envolvidas tanto em seu processo de compreensão quanto em sua gestão prática hoje. No entanto, apesar de ter uma incidência não desprezível e adquirir um papel central no manejo clínico da estenose aórtica, esta entidade parece ter passado de resistida para relativamente ignorada. Com o intuto de contribuir para sua visibilidade, esta revisão oferece um panorama abrangente do tema, revendo as principais contribuições históricas ao seu conhecimento e abordando em profundidade seus mecanismos, as chaves para sua detecção clínica e seu impacto na gestão da estenose aórtica e outras entidades com fisiopatologia relacionada.

Síndrome de Heyde / Heyde Syndrome

RESUMEN Introducción: El síndrome de Heyde es la asociación entre estenosis valvular aórtica y sangrado gastrointestinal, por angiodisplasia intestinal. La base fisiopatológica de ese síndrome parece ser una deficiencia adquirida del factor de Von Willebrand, que lleva al sangrado de malformaciones arteriovenosas angiodisplásicas. Las alternativas de tratamiento incluyen la localización de puntos de sangrado y la cauterización, pero tal opción terapéutica está asociada a alta recurrencia. El reemplazo de la válvula parece ofrecer la mejor esperanza de resolución a largo plazo del sangrado y debe ser considerada en la mayoría de las veces. Objetivos: Presentar el caso de un síndrome de Heyde, una causa infrecuente de hemorragia digestiva. Caso clínico: Paciente de 74 años con antecedentes estenosis aórtica y sangrado gastrointestinal debido a angiodisplasia duodenal localizada en la segunda porción de esta, tratada mediante la escleroterapia. Conclusiones: El síndrome de Heyde es una asociación poco frecuente entre una valvulopatía aortica y sangrado digestivo por una angiodisplasia que puede presentarse en cualquier parte del tubo digestivo(AU)

ABSTRACT Introduction: Heyde syndrome is the association between aortic valve stenosis and gastrointestinal bleeding, due to intestinal angiodysplasia. The pathophysiological basis of this syndrome seems to be acquired Von Willebrand factor deficiency, which leads to bleeding from angiodysplastic arteriovenous malformations. Management alternatives include localization of bleeding points and cauterization, but such a therapeutic option is associated with high recurrence. Valve replacement seems to offer the best hope for long-term solving of bleeding and should be considered for most of the cases. Objectives: To present a case of Heyde syndrome, a rare cause of gastrointestinal bleeding. Clinical case: A 74-year-old patient is presented with a history of aortic stenosis and gastrointestinal bleeding due to duodenal angiodysplasia located in the second portion of it, managed by sclerotherapy. Conclusions: Heyde syndrome is a rare association between aortic valve disease and digestive bleeding due to angiodysplasia that can occur in any part of the digestive tract(AU)

A Suspected Case of Heyde Syndrome with Bleeding of the Small Intestine before Aortic Valve Replacement for Severe Aortic Valve Stenosis / 日本心臓血管外科学会雑誌

The patient was a 74-year-old woman who had undergone mitral valve replacement with a mechanical valve for rheumatic mitral valve stenosis at age 60. She was scheduled for aortic valve replacement for severe aortic stenosis. However, she had significantly worsening anemia before the operation. Capsule endoscopy showed angiodysplasia with bleeding in her small intestine, which was considered the cause of the anemia. Because of progressive anemia, we tried embolization under angiography. However, there was no evidence of extravasation. Neither melena nor exacerbation of anemia was observed, and she underwent aortic valve replacement. She was discharged on postoperative day 22 without gastrointestinal bleeding. Heyde syndrome is aortic valve stenosis associated with gastrointestinal bleeding induced by von Willebrand disease and angiodysplasia in small intestine. Molecular multimeric analysis of von Willebrand factor and the existence of angiodysplasia with hemorrhage of the digestive tract are important for definitive diagnosis. Capsule endoscopy, which is a general examination, is more useful for diagnosis than molecular multimeric analysis of von Willebrand factor. Aortic valve replacement is the only therapeutic option for Heyde syndrome. It is important to decide the appropriate timing of AVR with cardiopulmonary bypass.

The clinical features of gastrointestinal bleeding complicating aortic stenosis / 中华内科杂志

Objective To deepen the understanding about Heyde's syndrome by investigating the clinical characteristics and prognosis of the patients with aortic valve stenosis complicating with gastrointestinal bleeding.Methods Patients with aortic valve stenosis and gastrointestinal bleeding coincidently admitted to our hospital from 2001 to 2011 were retrieved and analyzed.Results In all the 443 157 in-patients,474 patients were diagnosed with aortic valve stenosis (0.11％,474/443 157) and 14 patients (9 males and 5 females,aged 53-87 years old) with gastrointestinal bleeding coincidently (2.95 ％,14/474).Among the 14 patients,3 were moderate aortic valve stenosis,11 severe aortic valve stenosis.The aortic valve peak flow velocity was 324-709 (480.54 ± 188.25) cm/s and the mean aortic valve pressure gradient was 21.04-91.56 (56.93 ± 29.90) mm Hg (1 mm Hg =0.133 kPa).Heavy gastrointestinal bleeding was manifested in all the 14 patients with 1 of haematemesis and 13 of hematochezia.Hemoglobin (Hb) and red blood cell (RBC) count were significantly lower than the normal range [(69 ±28) g/L and (2.71 ±2.04) × 1012/L,P ＜0.05].Their mean corpuscular volume(MCV),mean corpuscular hemoglobin (MCH),mean corpuscular hemoglobin concentration (MCHC),platelet (PLT) count,prothrombin time (PT) and international normalized ratio (INR) were in normal range [(90.21 ± 2.94) r,(29.39 ± 1.99) pg,(327.57 ± 14.82) g/L,(185.13 ±22.55) × 109/L,(11.4 ± 1.04) s and 1.22 ±0.44,respectively].Among all the 14 patients,13 were over 65 years old and they all accepted gastrointestinal imaging (13/14).Vascular malformation of intestine was found in 6 patients with 4 lesions located in descending colon and 2 located in sigmoid colon.Hemorrhage foci were found in 2 patients with one of colon cancer,and another of duodenal ulcer,while no definite hemorrhage foci were found in the other 11 patients.A total of 6 patients with severe aortic valve stenosis underwent aortic valve replacement (AVR) successfully (6/11) and no recurrent gastrointestinal bleeding was ever found.Conservative treatment was performed in the other 5 patients with severe aortic valve stenosis (5/11) and resulted in sudden death in 2 patients (2/5).Conclusions Prompt echocardiography and gastrointestinal endoscopy should be performed in the elderly patients with obscure gastrointestinal bleeding to facilitate the early diagnosis and treatment of Heyde's syndrome.AVR is a fundamental procedure to improve the prognosis of Heyde's syndrome.

Heyde’s syndrome: Rarely heard and often missed.

Gastrointestinal blood loss is a common and important problem in clinical practice. It is sometimes difficult to locate and treat gastrointestinal bleeding. We discuss Heyde’s syndrome, an important cause of gastrointestinal bleeding in the elderly, in the context of a case report. Heyde’s syndrome is bleeding from gastrointestinal angiodysplasias in patients with aortic stenosis. For this, a wide range of investigations and treatment modalities are now available. One should therefore make an aggressive attempt to localise the bleeding site. Newer endoscopic technologies may prove beneficial. Aortic valve replacement is claimed to minimise or even stop the bleeding in such patients. But still there are a few reports showing gastrointestinal bleeding after aortic valve replacement. Old age and co-morbidities may create a hindrance in valve replacement or resection surgery. Some newer treatment options like hormonal and thalidomide therapy look promising but they have inadequate evidence behind them. Here, we discuss this clinical problem, strategies and evidence, areas of uncertainty, available guidelines, and our conclusions about Heyde’s syndrome.

A Case of Heyde's Syndrome with Abnormal von Willebrand Factor / 대한소화기학회지

A 68-year-old woman with known severe aortic stenosis was admitted to the hospital because of hematochezia and dizziness. She had received several blood transfusions over the preceding 3 years and undergone right hemicolectomy 2 years ago for severe lower gastrointestinal bleeding. Postoperative histology revealed angiodysplasia involving the ascending colon. After the hemicolectomy, she continued to have hematochezia and anemia and required additional blood transfusions for anemia. During this admission, platelet count, activated partial-thromboplastin time, von Willebrand factor antigen, and von Willebrand factor ristocetin cofactor were normal. She had a severe deficiency of high-molecular-weight multimers of von Willebrand factor. Colonoscopy showed angiodysplasia in the transverse colon at this time. Successful coagulation of the bleeding angiodysplasia was achieved by argon plasma coagulator. No additional bleeding was observed thereafter. We report a case of Heyde's syndrome with abnormal von Willebrand factor in a patient who presented with intestinal angiodysplasia and aortic stenosis.