Long Survival after Gastrointestinal Bleeding in a New-Onset Heyde’s Syndrome Patient Treated with Multiple Endoscopic Hemostatic Procedures and Repeated Transfusions after Aortic Valve Replacement

Heyde’s syndrome, which is caused by aortic stenosis and subsequent acquired von Willebrand factor deficiency, is a gastrointestinal bleeding disease. Gastrointestinal bleeding develops in patients with Heyde’s syndrome, which may have a different prognosis from general gastrointestinal bleeding; thus, it is important to understand the clinical course. We report a 76-year-old Japanese female who underwent aortic mechanical valve replacement 1 year ago and presented with recurrent gastrointestinal bleeding in angiodysplasia of the sigmoid colon. Endoscopic interventions achieved hemostasis. However, 6 rebleeding events occurred due to a sigmoid colon ulcer and gastric and jejunal angiodysplasia 7 years after first hemostasis. The patient underwent multiple endoscopic hemostatic procedures (upper, lower, and balloon-assisted endoscopy) and repeated transfusions (total of 394 units of red blood cells). The intensive treatment contributed to the survival time of 10 years. In addition, we performed a literature review of the prognosis of patients with Heyde’s syndrome.

Did the Angiodysplasia Associated With Heyde's Syndrome Disappear Spontaneously?: A Case Report

Background: Multidisciplinary clinical manifestations of Heyde's syndrome and finite accuracy of corresponding examinations toward Heyde's triad make it easily omitted or misjudged in practice. Moreover, aortic valve replacement is often delayed because of the contradiction between anticoagulation and hemostasis. Herein, we present a rare case of atypical Heyde's syndrome whose confirmed intermittent bleeding angiodysplasia was not observed via mesenteric arteriography again, but severe bleeding was dramatically improved by transcatheter aortic valve implantation(TAVI) following laparotomy.Case presentation: A 64-year-old female experienced refractory gastrointestinal bleeding and deteriorating exertional dyspnea with a history of hypertension. Exploratory laparotomy was performed because the hemorrhage persisted and repeated transfusions. The subsequent histological examination revealed angiodysplasia. Heyde's syndrome was not suspected until she bled again combined with aortic valve stenosis detected by echocardiography 3 years later. TAVI was consequently performed in a stable condition with the invisibility of angiodysplasia. The post-procedure and follow-up were uneventful.Conclusions: The visible figures of angiodysplasia or shortage of HMWM-vWFs should not be indispensable for the clinical diagnosis of Heyde's syndrome. Laparotomy could be a bridging therapy to aortic valve replacement for severe hemorrhagic patients, and TAVI may benefit high-risk patients with a stable condition.

Heyde’s syndrome

Gastrointestinal bleeding is a severe and potentially life-threatening condition associated with significant morbidity and mortality. In clinical practice, its differential dia­gnosis can sometimes be complicated, especially when bleeding is recurrent. One of the lesser-known reasons for recurrent gastrointestinal bleeding is the so called Heyde’s syndrome. It is a combination of bleeding from gastrointestinal angiodysplasia, aortic stenosis and acquired type 2A von Willebrand syndrome. In the submitted article, authors present a summary of the current knowledge on etiology and pathogenesis of the disease. The degenerative aortic valve disease, typically present in elderly patients, causes changes in the blood flow through stenotic opening. This results in elongation of otherwise globular von Willebrand’s factor (vWF) molecules, resulting in its reduced efficacy in hemostasis. According to new scientific discoveries, vWF also plays a role in angiogenesis and therefore in gastrointestinal angiodysplasia formation. This combination of factors results in recurrent bleeding typical for Heyde’s syndrome. Surgical aortic valve replacement is the gold standard treatment. A vicious circle can often be created between the gastroenterologist, who refers the patient for cardiac surgery procedures, and the cardiothoracic surgeon, who is waiting for a time when the patient will stop bleeding. A close communication and cooperation between the gastroenterologist, hematologist and cardiothoracic surgeon is required to properly manage the patient. The presence of angiodysplasia in an elderly patient, associated with recurrent gastrointestinal bleeding, should lead clinicians to look for aortic stenosis and consider Heyde’s syndrome as a potential dia­gnosis. Its interesting etiopathogenesis and rare occurrence led the authors to further investigate this topic. They also present a short summary of their own group of patients. Key words: Heyde’s syndrome – aortic stenosis – angiodysplasia – von Willebrand’s syndrome

Reappraising the spectrum of bleeding gastrointestinal angioectasia in a degenerative calcific aortic valve stenosis: Heyde’s syndrome

Background The occurrence of bleeding gastrointestinal angioectasia in elderly patients with degenerative calcific aortic stenosis is one of the most challenging clinical scenarios. A number of studies have shown that this clinical phenomenon is known as Heyde’s syndrome. Main body of the abstract The pathogenesis of Heyde’s syndrome is mainly due to the loss of high-molecular-weight von Willebrand factor (HMW vWF) multimers, as a consequent fragmentation of HMW vWF multimers as they pass through the stenosed aortic valve leading to acquired von Willebrand syndrome type IIA. Aortic valve replacement has proven to be a more effective management approach in the cessation of recurrent episodes of gastrointestinal bleeding. Short conclusion Physicians should have a high index of suspicion when dealing with elderly patients with established aortic stenosis presenting with iron deficiency anemia or unclear gastrointestinal bleeding. Parallel consultations between different specialties are essential for appropriate management.