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.

An elderly man with gastric cancer, Saint's triad and Heyde's syndrome.

This case study describes a 71-year-old man with signet-ring cell gastric adenocarcinoma and malignant sigmoidal polyp; and typical features of Saint's triad and Heyde syndrome. He had digestive bleeding, two types of hernia, diverticulosis, arterial hypertension, malignant polyp, and antecedent of smoking, lung tuberculosis, and surgical correction of aortic valve stenosis. There is a hypothetical inverse relationship between herniosis and development of malignancy; however, the patient herein described presented gastric and sigmoidal cancers. Gastrointestinal malignancies are sometimes associated with paraneoplastic entities, isolated or manifested as syndromes, but neither Saint's triad or Heyde syndrome have been included. This patient persisted clinically stable during the preoperative period, but suddenly died; Trousseau's syndrome would be the most probable mechanism of sudden death in this setting. Case reports can stimulate further studies to get additional knowledge about unusual entities.

An elderly man with gastric cancer, Saint's triad and Heyde's syndrome / Un anciano con cáncer gástrico, tríada de Saint y síndrome de Heyde

This case study describes a 71-year-old man with signet-ring cell gastric adenocarcinoma and malignant sigmoidal polyp; and typical features of Saint's triad and Heyde syndrome. He had digestive bleeding, two types of hernia, diverticulosis, arterial hypertension, malignant polyp, and antecedent of smoking, lung tuberculosis, and surgical correction of aortic valve stenosis. There is a hypothetical inverse relationship between herniosis and development of malignancy; however, the patient herein described presented gastric and sigmoidal cancers. Gastrointestinal malignancies are sometimes associated with paraneoplastic entities, isolated or manifested as syndromes, but neither Saint's triad or Heyde syndrome have been included. This patient persisted clinically stable during the preoperative period, but suddenly died; Trousseau's syndrome would be the most probable mechanism of sudden death in this setting. Case reports can stimulate further studies to get additional knowledge about unusual entities.

Este estudio de caso describe un hombre de 71 años de edad, con adenocarcinoma gástrico con células en anillo de sello y un pólipo maligno sigmoideo; y características típicas de la tríada de Saint y del síndrome de Heyde. Tuvo una hemorragia digestiva, dos tipos de hernias, divertículos, hipertensión arterial, y pólipo maligno; con antecedente de tabaquismo, tuberculosis pulmonar, y corrección quirúrgica de estenosis de la válvula aórtica. Hay una hipotética relación inversa entre hernioses y el desarrollo de malignidades; sin embargo, el paciente que se describe en el presente documento presentó cánceres gástrico y sigmoideo. Neoplasias gastrointestinales se asocian a veces con entidades para neoplásicas aisladas o manifiestan síndromes, pero ni la tríada de Saint ni el síndrome de Heyde se ha incluido. Este paciente persistió clínicamente estable durante el período preoperatorio, pero de repente murió; síndrome de Trousseau sería el mecanismo más probable de muerte súbita en esta situación. Los informes de casos pueden estimular más estudios para obtener un conocimiento adicional sobre esas entidades inusuales.

Acquired Von Willebrand Syndrome In Aortic Stenosis: Case Report And Review.

We present a case of a 61 year old man who presented with a 3 week history of easy bruising and ecchymoses in both thighs and right arm without significant trauma. Physical exam was remarkable for oozing gums, diminished second heart sound, a systolic ejection murmur at the aortic position with radiation to carotids, and delayed pulses. Laboratories were remarkable for iron deficiency anemia. Echocardiogram was consistent with severe aortic stenosis. Colonoscopy revealed several arteriovenous malformations throughout the colon. There is an association between severe aortic stenosis and gastrointestinal bleeding. The pathogenesis of Heyde's Syndrome involves iron deficiency anemia due to acquired von Willebrand factor (vWF) deficiency and ultimately gastrointestinal angiodysplasia. Correct diagnosis and management warrants a multidisciplinary approach.

Prevalence, variability, and outcomes in portal hypertensive colopathy: a study in patients with cirrhosis and paired controls.

BACKGROUND: Management of portal hypertensive colopathy (PHC) has been challenged by controversial results in its prevalence and clinical relevance. OBJECTIVE: To describe the PHC prevalence and to evaluate the variability in diagnosis, the relation to severity of liver disease, and the incidence of severe outcomes. DESIGN: Cross-sectional study. SETTING: Endoscopic unit of a tertiary-care academic center in Rio de Janeiro, Brazil. PATIENTS: Patients with cirrhosis with portal hypertension and controls paired for age and sex. INTERVENTIONS: All patients were submitted to standard and image-enhanced colonoscopies, which were recorded in a coded video file and analyzed twice by a blinded endoscopist. MAIN OUTCOME MEASUREMENTS: The prevalence of PHC. RESULTS: A total of 51 patients with cirrhosis (55% male, mean age 59 years) and 51 healthy controls (43% male, mean age 61 years) were included. The top ranking colonoscopic findings were angiodysplasia-like lesions, nonspecific vascular pattern, red spots, and colorectal varices, all significantly more frequent in patients with cirrhosis compared with controls. PHC prevalence was 71% in patients with cirrhosis. For PHC, interobserver and intraobserver agreement (k values [standard error]) were 0.68 (0.09) and 0.63 (0.10), respectively. Intraobserver agreement for colonoscopic findings was satisfactory. PHC was not related to more severe liver disease or liver stiffness. Only 5 patients developed severe outcomes during follow-up. LIMITATIONS: The exclusion of patients with cirrhosis without esophageal varices and the absence of an interobserver agreement analysis by double-blinded endoscopists. CONCLUSION: PHC was highly prevalent in patients with cirrhosis, and its diagnostic agreement was satisfactory. PHC is not associated with relevant severe outcomes in a 12-month follow-up.

Heyde syndrome in a 71-year-old man who underwent chest radiotherapy at young age.

We report the case of a 71-year-old man with diagnosis of aortic valve stenosis for ten years, who came to hospital because of breathlessness during the previous two months and recent low intestinal hemorrhage. On admission, laboratory tests and upper gastrointestinal endoscopy and colonoscopy revealed anemia and bleeding cecal angiodysplasia. The echocardiography study showed a severe aortic stenosis. Classical Heyde syndrome is described as the association of aortic stenosis, bleeding gastrointestinal angiodysplasia and secondary anemia. The antecedent of mediastinal radiotherapy for treatment of Hodgkin's disease during his youth, and eventual late cardiac adverse effects that may include aortic or mitral valve disturbances are highlighted. Electrocoagulation with argonium was performed on the sites of active bleeding during the colonoscopy. In sequence, surgical replacement by bioprothesis was done on the aortic valve. The patient remains asymptomatic, under long-term outpatient surveillance, with normal control evaluations. The aim of this case study is to emphasize difficulties related to diagnosis, and to highlight the role of endoscopy and imaging studies to confirm a hypothesis of this underestimated condition.

Ectasia vascular como causa de hemorragia digestiva: un peligro latente / Vascular ectasia as cause digestive hemorrhage: a latent danger

Se presentan tres casos de hemorragia digestiva producidas por ectasias vasculares localizadas a nivel de colon en los tres pacientes, presentandose simultaneamente en una de ellos en el estomago. Se ilustran los métodos de investigación mas apropiados para el diagnóstico precoz de las mismas. Se alerta enfáticamente al médico sobre la necesidad de estudiar profundamente las anemias crónicas en pacientes ancianos (de localización desconocida). Se actualiza en cuanto a definición, fisiopatología, métodos de estudio y tratamiento de las ectasias vasculares, como así tambien sus complicaciones.

Ectasia vascular como causa de hemorragia digestiva: un peligro latente / Vascular ectasia as cause digestive hemorrhage: a latent danger

Se presentan tres casos de hemorragia digestiva producidas por ectasias vasculares localizadas a nivel de colon en los tres pacientes, presentandose simultaneamente en una de ellos en el estomago. Se ilustran los métodos de investigación mas apropiados para el diagnóstico precoz de las mismas. Se alerta enfáticamente al médico sobre la necesidad de estudiar profundamente las anemias crónicas en pacientes ancianos (de localización desconocida). Se actualiza en cuanto a definición, fisiopatología, métodos de estudio y tratamiento de las ectasias vasculares, como así tambien sus complicaciones. (AU)

Angiodisplasia gástrica / Gastric angiodysplasia

La angiodisplasia es un trastorno vascular poco frecuente, siendo la causa de hemorragias digestivas crónicas o recurrentes que llevan a la realización de estudios frecuentemente negativos. Las localizaciones en colon ascendente y ciego son las mas comunes, mientras que las de estómago e intestino delgado son inhabituales. La etiología es aún desconocida, pero actualmente existen varias teorías que intentan explicar su mecanismo de formación. Las imágenes radiológicas al igual que las endoscópicas son inespecíficas y en el último caso, a veces pueden simular áreas de gastritis o lesiones por traumatismo endoscópico. Dado que las lesiones son generalmente submucosas, el método de estudio ideal es la angiografía. Es mucho mas común en personas añosas, existiendo también la relación con una valvulopatía aórtica, aunque también ha sido descripta en sujetos mas jóvenes. El tratamiento puede ser quirúrgico o por electrocoagulación con una muy baja morbimortalidad. La mayor parte de la literatura mundial de los últimos tres años, está referida fundamentalmente a la localización colónica, dada la gran rareza de la ubicación gástrica.

Angiodisplasia gástrica / Gastric angiodysplasia

La angiodisplasia es un trastorno vascular poco frecuente, siendo la causa de hemorragias digestivas crónicas o recurrentes que llevan a la realización de estudios frecuentemente negativos. Las localizaciones en colon ascendente y ciego son las mas comunes, mientras que las de estómago e intestino delgado son inhabituales. La etiología es aún desconocida, pero actualmente existen varias teorías que intentan explicar su mecanismo de formación. Las imágenes radiológicas al igual que las endoscópicas son inespecíficas y en el último caso, a veces pueden simular áreas de gastritis o lesiones por traumatismo endoscópico. Dado que las lesiones son generalmente submucosas, el método de estudio ideal es la angiografía. Es mucho mas común en personas añosas, existiendo también la relación con una valvulopatía aórtica, aunque también ha sido descripta en sujetos mas jóvenes. El tratamiento puede ser quirúrgico o por electrocoagulación con una muy baja morbimortalidad. La mayor parte de la literatura mundial de los últimos tres años, está referida fundamentalmente a la localización colónica, dada la gran rareza de la ubicación gástrica.(AU)