Heyde’s syndrome

2021 ◽  
Vol 75 (4) ◽  
pp. 298-303
Author(s):  
Renáta Michalová ◽  
Radoslava Šimonová ◽  
Renáta Michalová ml. ◽  
Peter Bánovčin ◽  
Rudolf Hyrdel

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

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 1403-1403
Author(s):  
Pernilla D'Souza ◽  
Mark Blostein

Abstract Abstract 1403 In 1958, EC Heyde described a syndrome of iron deficiency anemia due to gastrointestinal bleeding (GI) in a patient with calcific aortic stenosis. In 1992, Warkentin et al. hypothesized a bleeding diathesis due to a link between Heyde's Syndrome and acquired Von Willebrand Syndrome. This bleeding syndrome has now been shown to result from the loss of the largest multimers of von Willebrand Factor (VWF) and is therefore classified as acquired Type 2A Von Willebrand Syndrome. Hypotheses suggest that the high shear stresses that are obtained in tortuous angiodysplastic lesions work with this deficiency of VWF, to produce gastrointestinal bleeding that is notoriously difficult to elucidate on endoscopy. It has been shown repeatedly that replacement of a stenotic aortic valve results in cessation of bleeding. Here we present a case of Heyde's Syndrome diagnosed with abnormal Closure Times and normal VWF Ristocetin cofactor activity. In this case, a 79-year-old man with known aortic stenosis and several episodes of GI bleeding was cured of a life threatening hemorrhage after the replacement of his stenotic aortic valve. At the time of his first notable gastrointestinal bleed, a tagged RBC scan showed a source of hemorrhage in the small bowel. Subsequently, two video capsule endoscopies showed jejunal angiodysplasia. After recurrent bleeding episodes, this patient presented with a life-threatening GI hemorrhage, which, in the context of aortic stenosis, raised the suspicion for Heyde's Syndrome. At this time, he presented with hematochezia requiring massive transfusions, and admission to the Intensive Care Unit. A tagged RBC scan showed active bleeding in a location that matched previous scans. The following tests were within normal limits: Factor VIII (1.53), VWF Ag (1.26), VWF:Rco activity (1.11), and the ratio of VWF Ag/VWF:Rco (0.88). However, the Dade Behring PFA-100 platelet function analyzer demonstrated that Closure Times with collagen/adenosine (> 300 sec) and with collagen/epinephrine (> 300 sec) were prolonged. In the clinical context consistent with Heyde's Syndrome, the patient's native aortic valve was replaced with a 21mm Carpentier-Edwards Magna Ease Bovine valve. As is classic for this syndrome, the valve replacement was curative. Since the surgery, the patient has not required further transfusions or interventions for gastrointestinal hemorrhage. In this case, our observations are consistent with previous reports by Warkentin et al. and Vincentellli et al. What is unique about our current report is that we measured both Ristocetin cofactor activity and Closure Times, two commonly available assays in most coagulation laboratories. Ristocetin cofactor activity is the current gold standard for measuring platelet function activity but may miss activities under high shear stress. The Closure Time, on the other hand, is able to detect defects in platelet aggregation under such conditions and may be the only manner by which such abnormalities in VWF function are detected. Therefore, we conclude that Closure Times should be used to screen for acquired Von Willebrand's Syndrome in Heyde's Syndrome. Disclosures: No relevant conflicts of interest to declare.


2021 ◽  
Vol 33 (1) ◽  
Author(s):  
Nelson Musilanga ◽  
Zhou Hongli ◽  
Cai Hongyu

Abstract 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.


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Charbel Abi Khalil ◽  
Barbara Ignatiuk ◽  
Guliz Erdem ◽  
Hiam Chemaitelly ◽  
Fabio Barilli ◽  
...  

AbstractTranscatheter aortic valve replacement (TAVR) has shown to reduce mortality compared to surgical aortic valve replacement (sAVR). However, it is unknown which procedure is associated with better post-procedural valvular function. We conducted a meta-analysis of randomized clinical trials that compared TAVR to sAVR for at least 2 years. The primary outcome was post-procedural patient-prosthesis-mismatch (PPM). Secondary outcomes were post-procedural and 2-year: effective orifice area (EOA), paravalvular gradient (PVG) and moderate/severe paravalvular leak (PVL). We identified 6 trials with a total of 7022 participants with severe aortic stenosis. TAVR was associated with 37% (95% CI [0.51–0.78) mean RR reduction of post-procedural PPM, a decrease that was not affected by the surgical risk at inclusion, neither by the transcatheter heart valve system. Postprocedural changes in gradient and EOA were also in favor of TAVR as there was a pooled mean difference decrease of 0.56 (95% CI [0.73–0.38]) in gradient and an increase of 0.47 (95% CI [0.38–0.56]) in EOA. Additionally, self-expandable valves were associated with a higher decrease in gradient than balloon ones (beta = 0.38; 95% CI [0.12–0.64]). However, TAVR was associated with a higher risk of moderate/severe PVL (pooled RR: 9.54, 95% CI [5.53–16.46]). All results were sustainable at 2 years.


Open Heart ◽  
2021 ◽  
Vol 8 (1) ◽  
pp. e001443
Author(s):  
Richard Paul Steeds ◽  
David Messika-Zeitoun ◽  
Jeetendra Thambyrajah ◽  
Antonio Serra ◽  
Eberhard Schulz ◽  
...  

AimsThere is an increasing awareness of gender-related differences in patients with severe aortic stenosis and their outcomes after surgical aortic valve replacement (SAVR) and transcatheter aortic valve implantation (TAVI).MethodsData from the IMPULSE registry were analysed. Patients with severe aortic stenosis (AS) were enrolled between March 2015 and April 2017 and stratified by gender. A subgroup analysis was performed to assess the impact of age.ResultsOverall, 2171 patients were enrolled, and 48.0% were female. Women were characterised by a higher rate of renal impairment (31.7 vs 23.3%; p<0.001), were at higher surgical risk (EuroSCORE II: 4.5 vs 3.6%; p=0.001) and more often in a critical preoperative state (7.0vs 4.2%; p=0.003). Men had an increased rate of previous cardiac surgery (9.4 vs 4.7%; p<0.001) and a reduced left ventricular ejection fraction (4.9 vs 1.3%; p<0.001). Concomitant mitral and tricuspid valve disease was substantially more common among women. Symptoms were highly prevalent in both women and men (83.6 vs 77.3%; p<0.001). AVR was planned in 1379 cases. Women were more frequently scheduled to undergo TAVI (49.3 vs 41.0%; p<0.001) and less frequently for SAVR (20.3 vs 27.5%; p<0.001).ConclusionsThe present data show that female patients with severe AS have a distinct patient profile and are managed in a different way to males. Gender-based differences in the management of patients with severe AS need to be taken into account more systematically to improve outcomes, especially for women.


Author(s):  
Felipe Homem Valle ◽  
Fernando Pivatto Júnior ◽  
Luiz Carlos Corsetti Bergoli ◽  
Rodrigo Vugman Wainstein ◽  
Marco Vugman Wainstein

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