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Maksić M, Corović I, Stanisavljević I, Radojević D, Veljković T, Todorović Ž, Jovanović M, Zdravković N, Stojanović B, Marković BS, Jovanović I. Heyde Syndrome Unveiled: A Case Report with Current Literature Review and Molecular Insights. Int J Mol Sci 2024; 25:11041. [PMID: 39456826 PMCID: PMC11507012 DOI: 10.3390/ijms252011041] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/08/2024] [Revised: 09/23/2024] [Accepted: 09/26/2024] [Indexed: 10/28/2024] Open
Abstract
Heyde syndrome, marked by aortic stenosis, gastrointestinal bleeding from angiodysplasia, and acquired von Willebrand syndrome, is often underreported. Shear stress from a narrowed aortic valve degrades von Willebrand factor multimers, leading to angiodysplasia formation and von Willebrand factor deficiency. This case report aims to raise clinician awareness of Heyde syndrome, its complexity, and the need for a multidisciplinary approach. We present a 75-year-old man with aortic stenosis, gastrointestinal bleeding from angiodysplasia, and acquired von Willebrand syndrome type 2A. The patient was successfully treated with argon plasma coagulation and blood transfusions. He declined further treatment for aortic stenosis but was in good overall health with improved laboratory results during follow-up. Additionally, we provide a comprehensive review of the molecular mechanisms involved in the development of this syndrome, discuss current diagnostic and treatment approaches, and offer future perspectives for further research on this topic.
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Affiliation(s)
- Mladen Maksić
- Department of Internal Medicine, Faculty of Medical Sciences, University of Kragujevac, Svetozara Markovica 69, 34000 Kragujevac, Serbia; (M.M.); (D.R.); (Ž.T.); (M.J.); (N.Z.)
| | - Irfan Corović
- Center for Molecular Medicine and Stem Cell Research, Faculty of Medical Sciences, University of Kragujevac, Svetozara Markovica 69, 34000 Kragujevac, Serbia; (I.C.); (I.S.); (I.J.)
| | - Isidora Stanisavljević
- Center for Molecular Medicine and Stem Cell Research, Faculty of Medical Sciences, University of Kragujevac, Svetozara Markovica 69, 34000 Kragujevac, Serbia; (I.C.); (I.S.); (I.J.)
| | - Dušan Radojević
- Department of Internal Medicine, Faculty of Medical Sciences, University of Kragujevac, Svetozara Markovica 69, 34000 Kragujevac, Serbia; (M.M.); (D.R.); (Ž.T.); (M.J.); (N.Z.)
| | - Tijana Veljković
- Department of Pediatrics, Faculty of Medical Sciences, University of Kragujevac, Svetozara Markovica 69, 34000 Kragujevac, Serbia;
| | - Željko Todorović
- Department of Internal Medicine, Faculty of Medical Sciences, University of Kragujevac, Svetozara Markovica 69, 34000 Kragujevac, Serbia; (M.M.); (D.R.); (Ž.T.); (M.J.); (N.Z.)
| | - Marina Jovanović
- Department of Internal Medicine, Faculty of Medical Sciences, University of Kragujevac, Svetozara Markovica 69, 34000 Kragujevac, Serbia; (M.M.); (D.R.); (Ž.T.); (M.J.); (N.Z.)
- Center for Molecular Medicine and Stem Cell Research, Faculty of Medical Sciences, University of Kragujevac, Svetozara Markovica 69, 34000 Kragujevac, Serbia; (I.C.); (I.S.); (I.J.)
| | - Nataša Zdravković
- Department of Internal Medicine, Faculty of Medical Sciences, University of Kragujevac, Svetozara Markovica 69, 34000 Kragujevac, Serbia; (M.M.); (D.R.); (Ž.T.); (M.J.); (N.Z.)
| | - Bojan Stojanović
- Department of Surgery, Faculty of Medical Sciences, University of Kragujevac, Svetozara Markovica 69, 34000 Kragujevac, Serbia;
| | - Bojana Simović Marković
- Center for Molecular Medicine and Stem Cell Research, Faculty of Medical Sciences, University of Kragujevac, Svetozara Markovica 69, 34000 Kragujevac, Serbia; (I.C.); (I.S.); (I.J.)
| | - Ivan Jovanović
- Center for Molecular Medicine and Stem Cell Research, Faculty of Medical Sciences, University of Kragujevac, Svetozara Markovica 69, 34000 Kragujevac, Serbia; (I.C.); (I.S.); (I.J.)
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Sugino S, Inoue K, Zen K, Yashige M, Kobayashi R, Takamatsu K, Ito N, Iwai N, Hirose R, Doi T, Dohi O, Yoshida N, Uchiyama K, Takagi T, Ishikawa T, Konishi H, Matoba S, Itoh Y. Gastrointestinal Angiodysplasia in Patients with Severe Aortic Stenosis: The Endoscopic Features of Heyde's Syndrome. Digestion 2023; 104:468-479. [PMID: 37619533 DOI: 10.1159/000533237] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/08/2023] [Accepted: 07/22/2023] [Indexed: 08/26/2023]
Abstract
INTRODUCTION Aortic stenosis (AS) is sometimes associated with gastrointestinal bleeding, and this phenomenon is known as Heyde's syndrome. Such bleeding is most often considered to originate from gastrointestinal angiodysplasias, but the frequency and endoscopic features of such bleeding remain unclear. This study aimed to determine the frequency and endoscopic features of gastrointestinal angiodysplasia in patients with severe AS. PATIENTS AND METHODS In this multicenter, retrospective study, we evaluated consecutive patients who underwent transcatheter aortic valve implantation (TAVI) with severe AS from May 2016 to December 2019. We extracted the data on the clinicopathological features according to the status of anemia, the proportion of patients who underwent gastrointestinal endoscopic examinations and demonstrated gastrointestinal angiodysplasia, and identified the endoscopic features associated with such patients. RESULTS In 325 patients, the rates of moderate/severe anemia (hemoglobin < 11 g/dL) were 52%. Regarding medicine, there were no significant differences between the patients with and without moderate/severe anemia. Patients were examined by esophagogastroduodenoscopy (21%), colonoscopy (12%), and balloon-assisted enteroscopy or small bowel capsule endoscopy (1.5%). Patients with moderate/severe anemia had significantly more angiodysplasia (38.3% vs. 7.7%; p < 0.0001) and active bleeding (23.4% vs. 0%; p < 0.01). Angiodysplasia was detected in 21 patients (stomach, n = 9; small intestine, n = 5, and colon, n = 10). CONCLUSIONS The results suggest, for the first time, that patients with severe AS who underwent TAVI and moderate/severe anemia frequently had gastrointestinal angiodysplasia and active bleeding throughout the entire gastrointestinal tract.
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Affiliation(s)
- Satoshi Sugino
- Department of Molecular Gastroenterology and Hepatology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan,
- Department of Gastroenterology and Hepatology, Asahi University Hospital, Gifu, Japan,
| | - Ken Inoue
- Department of Molecular Gastroenterology and Hepatology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan
| | - Kan Zen
- Department of Cardiovascular Medicine, Kyoto Prefectural University of Medicine Graduate School of Medical Science, Kyoto, Japan
| | - Masaki Yashige
- Department of Cardiovascular Medicine, Kyoto Prefectural University of Medicine Graduate School of Medical Science, Kyoto, Japan
| | - Reo Kobayashi
- Department of Molecular Gastroenterology and Hepatology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan
| | - Kazuaki Takamatsu
- Department of Cardiovascular Medicine, Kyoto Prefectural University of Medicine Graduate School of Medical Science, Kyoto, Japan
| | - Nobuyasu Ito
- Department of Cardiovascular Medicine, Kyoto Prefectural University of Medicine Graduate School of Medical Science, Kyoto, Japan
| | - Naoto Iwai
- Department of Molecular Gastroenterology and Hepatology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan
| | - Ryohei Hirose
- Department of Molecular Gastroenterology and Hepatology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan
| | - Toshifumi Doi
- Department of Molecular Gastroenterology and Hepatology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan
| | - Osamu Dohi
- Department of Molecular Gastroenterology and Hepatology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan
| | - Naohisa Yoshida
- Department of Molecular Gastroenterology and Hepatology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan
| | - Kazuhiko Uchiyama
- Department of Molecular Gastroenterology and Hepatology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan
| | - Tomohisa Takagi
- Department of Molecular Gastroenterology and Hepatology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan
| | - Takeshi Ishikawa
- Department of Molecular Gastroenterology and Hepatology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan
| | - Hideyuki Konishi
- Department of Molecular Gastroenterology and Hepatology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan
| | - Satoaki Matoba
- Department of Cardiovascular Medicine, Kyoto Prefectural University of Medicine Graduate School of Medical Science, Kyoto, Japan
| | - Yoshito Itoh
- Department of Molecular Gastroenterology and Hepatology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan
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Wang L, Wu K, Ou W, Su X, Sun G, Wang W, Xu Q, Wang B. Transcatheter aortic valve implantation for patients with heyde syndrome: A literature review of case reports. Heliyon 2023; 9:e17952. [PMID: 37539190 PMCID: PMC10395290 DOI: 10.1016/j.heliyon.2023.e17952] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/21/2023] [Revised: 06/28/2023] [Accepted: 07/03/2023] [Indexed: 08/05/2023] Open
Abstract
Objective A systematic review of international case reports of patients with Heyde syndrome (HS) treated by transcatheter aortic valve implantation (TAVI) was conducted to explore the clinical characteristics of this group of patients and sirgical success. Methods: Electronic databases, including PubMed, Embase and CNKI, were searched with combinations of the search terms, Heyde syndrome, gastrointestinal bleeding, aortic stenosis, angiodysplasia and transcatheter aortic valve replacement. All case reports were screened according to inclusion criteria, and HS patient data was summarized. Results A total of 31 case reports concerned patients with a history of aortic stenosis and repeated gastrointestinal bleeding. Ultrasonic cardiograms (UCG) were recorded for 27 cases, including those with critical aortic stenosis (n = 26). Gastrointestinal sequelae were reported in 22 cases with duodenal and jejunal being the most common (n = 9). High-molecular-weight multimers of von Willebrand Factor (vWF-HMWM) were measured in 17 cases with the majority being lower (n = 15) and the minority normal (n = 2). All patients experienced recurrent bleeding after medication and endoscopic therapy and symptoms improved after TAVI (31/31). vWF was at normal levels in 11/12 cases post-TAVI. Twenty-five patients were followed up and 22 had no recurrence of symptoms giving an efficacy rate of 88% for TAVI in HS patients. Conclusions HS is characterized by angiodysplasia, aortic stenosis and von Willebrand disease with frequent recurrence of bleeding after drug and endoscopic treatment. TAVI is an effective therapy with an 88% resolution rate.
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Affiliation(s)
- Lilan Wang
- School of Medicine, Xiamen University. Xiamen 361005, China. Xiamen Cardiovascular Hospital, Xiamen University, Xiamen, 361000, China
| | - Kaimin Wu
- Xiamen Cardiovascular Hospital, Xiamen University, Xiamen, 361000, China
| | - Weimei Ou
- Xiamen Cardiovascular Hospital, Xiamen University, Xiamen, 361000, China
| | - Xin Su
- Xiamen Cardiovascular Hospital, Xiamen University, Xiamen, 361000, China
| | - Guangfeng Sun
- Xiamen Cardiovascular Hospital, Xiamen University, Xiamen, 361000, China
| | - Weimin Wang
- School of Medicine, Xiamen University. Xiamen 361005, China. Xiamen Cardiovascular Hospital, Xiamen University, Xiamen, 361000, China
| | - Qiaoru Xu
- School of Medicine, Xiamen University. Xiamen 361005, China. Xiamen Cardiovascular Hospital, Xiamen University, Xiamen, 361000, China
| | - Bin Wang
- School of Medicine, Xiamen University. Xiamen 361005, China. Xiamen Cardiovascular Hospital, Xiamen University, Xiamen, 361000, China
- Xiamen Cardiovascular Hospital, Xiamen University, Xiamen, 361000, China
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Lu C, Zhang Y. Gastrointestinal bleeding during the transcatheter aortic valve replacement perioperative period: A Review. Medicine (Baltimore) 2022; 101:e31953. [PMID: 36482568 PMCID: PMC9726417 DOI: 10.1097/md.0000000000031953] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/13/2022] Open
Abstract
With the aging of the population, the incidence of senile degenerative valvular heart disease is expected to increase. Transcatheter aortic valve replacement (TAVR) has been used for patients at lower surgical risk with symptomatic severe aortic valve stenosis. Because of the improvements in TAVR technology and increasing experience of the operators, TAVR is regarded as a safe and feasible procedure. Bleeding events during the TAVR perioperative period, especially gastrointestinal (GI) bleeding, have been proven to be related to the long-term prognosis and mortality. Elderly patients with valvular heart disease are susceptible to GI bleeding because of their use of antithrombotic drugs, physical damage of coagulation factors, and GI angiodysplasia. Frequent GI bleeding and low levels of preoperative hemoglobin increase the risk of TAVR, especially for elderly patients. Because of these risks, which are easily overlooked, we should focus more attention on the perioperative management of TAVR. Reasonable screening tools, including blood examinations, risk evaluation scales, and endoscopy, are beneficial to the prevention of complications that can occur during the perioperative period. Additionally, medical therapy can safely help patients at high-risk for bleeding patients throughout the perioperative period. This study aimed to characterize the pathology of TAVR patients and discuss treatment strategies for GI bleeding during the perioperative period.
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Affiliation(s)
- Chuan Lu
- From the Second Hospital of Dalian Medicial University, Shahekou District, Dalian City, China
| | - Yue Zhang
- From the Second Hospital of Dalian Medicial University, Shahekou District, Dalian City, China
- * Correspondence: Yue Zhang, From the Second Hospital of Dalian Medicial University, 467 Zhongshan Road, Shahekou District, Dalian city, China (e-mail: )
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Ramesh P, Kanagalingam S, Zargham Ul Haq F, Victory Srinivasan N, Khan AI, Mashat GD, Hazique M, Khan KI, Khan S. Acquired Von Willebrand Deficiency in Adults With Aortic Stenosis: A Systematic Review. Cureus 2022; 14:e28879. [PMID: 36225403 PMCID: PMC9541123 DOI: 10.7759/cureus.28879] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/11/2022] [Accepted: 09/07/2022] [Indexed: 11/16/2022] Open
Abstract
Von Willebrand factor (VWF) deficiency is associated with bleeding complications. The congenital type of Von Willebrand disease(VWD) is a very well-known bleeding disorder and sometimes may be associated with life-threatening hemorrhage. This systematic review is aimed at gathering further knowledge regarding the pathology of an acquired VWD form within a population of patients with aortic stenosis (AS) by shortlisting quality articles on this theme, through the Preferred Reporting Items for Systematic Review and Meta-analysis (PRISMA) 2020 guidelines. High shear stress caused by the stenotic valve cleaves VWF multimers, causing a relative state of deficiency. The condition returns to baseline immediately following surgical replacement of the valve. Results across eight studies reviewed by a majority concluded that in an AS patient with bleeding, the most likely cause is an acquired deficiency of VWF, associated with factors influencing blood flow and caused by the in-situ valve. However, several studies suggested otherwise/were misclassifications. This review highlighted the relationship between AS and acquired VWF deficiency and should be foreseen as an adverse complication, attracting further research and future theragnostic strategies for this condition.
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Affiliation(s)
- Prasana Ramesh
- Internal Medicine, California Institute of Behavioral Neurosciences & Psychology, Fairfield, USA
| | | | - Fnu Zargham Ul Haq
- Internal Medicine, California Institute of Behavioral Neurosciences & Psychology, Fairfield, USA
| | | | - Aujala Irfan Khan
- Research, California Institute of Behavioral Neurosciences & Psychology, Fairfield, USA
| | - Ghadi D Mashat
- Pediatrics, California Institute of Behavioral Neurosciences & Psychology, Fairfield, USA
| | - Mohammad Hazique
- Internal Medicine, California Institute of Behavioral Neurosciences & Psychology, Fairfield, USA
| | - Kokab Irfan Khan
- Research, California Institute of Behavioral Neurosciences & Psychology, Fairfield, USA
| | - Safeera Khan
- Internal Medicine, California Institute of Behavioral Neurosciences & Psychology, Fairfield, USA
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Saha B, Wien E, Fancher N, Kahili-Heede M, Enriquez N, Velasco-Hughes A. Heyde's syndrome: a systematic review of case reports. BMJ Open Gastroenterol 2022; 9:bmjgast-2021-000866. [PMID: 35534046 PMCID: PMC9086603 DOI: 10.1136/bmjgast-2021-000866] [Citation(s) in RCA: 12] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/20/2021] [Accepted: 03/27/2022] [Indexed: 11/12/2022] Open
Abstract
Objective Heyde’s syndrome (HS), a rare condition characterised by a unique relationship between severe aortic stenosis and angiodysplasia, is often diagnosed late increasing the risk for a prolonged hospital course and mortality in the elderly. The leading hypothesis explaining the aetiology of HS is acquired von Willebrand syndrome (AVWS) but not all studies support this claim. While individual cases of HS have been reported, here we present the first systematic review of case reports and focus on the prevalence of AVWS. Design A systematic search was conducted through PubMed/MEDLINE, CINAHL-EBSCO, Web of Science and Google Scholar since inception. The resulting articles were screened by two independent reviewers based on inclusion criteria that the article must be a case report/series or a letter to the editor in English describing HS in an adult patient. Results Seventy-four articles encompassing 77 cases met the inclusion criteria. The average age was 74.3±9.3 years old with a slight female predominance. The small intestine, especially the jejunum, was the most common location for bleeding origin. Capsule endoscopy and double balloon enteroscopy were superior at identifying bleeding sources than colonoscopy (p=0.0027 and p=0.0095, respectively) and oesophagogastroduodenoscopy (p=0.0006 and p=0.0036, respectively). The mean duration from symptom onset to diagnosis/treatment of HS was 23.8±39 months. Only 27/77 cases provided evidence for AVWS. Surgical and transcutaneous aortic valve replacement (AVR) were superior at preventing rebleeding than non-AVR modalities (p<0.0001). Conclusion Further research is warranted for a stronger understanding and increased awareness of HS, which may hasten diagnosis and optimal management.
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Affiliation(s)
- Bibek Saha
- John A. Burns School of Medicine, University of Hawai'i at Mānoa, Honolulu, Hawaii, USA
| | - Eric Wien
- John A. Burns School of Medicine, University of Hawai'i at Mānoa, Honolulu, Hawaii, USA.,Internal Medicine Residency Program, University of Hawai'i at Mānoa, Honolulu, Hawaii, USA
| | - Nicholas Fancher
- John A. Burns School of Medicine, University of Hawai'i at Mānoa, Honolulu, Hawaii, USA
| | - Melissa Kahili-Heede
- John A. Burns School of Medicine, University of Hawai'i at Mānoa, Honolulu, Hawaii, USA
| | - Nathaniel Enriquez
- John A. Burns School of Medicine, University of Hawai'i at Mānoa, Honolulu, Hawaii, USA.,Internal Medicine Residency Program, University of Hawai'i at Mānoa, Honolulu, Hawaii, USA
| | - Alena Velasco-Hughes
- John A. Burns School of Medicine, University of Hawai'i at Mānoa, Honolulu, Hawaii, USA.,Queen's Medical Group Hospitalist Program, Queen's Medical Center, Honolulu, Hawaii, USA
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Musilanga N, Hongli Z, Hongyu C. Reappraising the spectrum of bleeding gastrointestinal angioectasia in a degenerative calcific aortic valve stenosis: Heyde’s syndrome. THE EGYPTIAN JOURNAL OF INTERNAL MEDICINE 2021. [DOI: 10.1186/s43162-021-00046-z] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/10/2022] Open
Abstract
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.
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Notoya G, Niikura R, Yamada A, Ochi M, Kawai T, Koike K. 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. Case Rep Gastroenterol 2021; 15:819-824. [PMID: 34720829 PMCID: PMC8543306 DOI: 10.1159/000519004] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/12/2021] [Accepted: 07/30/2021] [Indexed: 11/21/2022] Open
Abstract
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.
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Affiliation(s)
- Genso Notoya
- Department of Gastroenterology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Ryota Niikura
- Department of Gastroenterology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan.,Gastroenterological Endoscopy, Tokyo Medical University, Tokyo, Japan
| | - Atsuo Yamada
- Department of Gastroenterology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Masanori Ochi
- Department of Gastroenterology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Takashi Kawai
- Gastroenterological Endoscopy, Tokyo Medical University, Tokyo, Japan
| | - Kazuhiko Koike
- Department of Gastroenterology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
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Al-Radaideh O, Farouji I, Abed H, Shaaban H. Recurrent gastrointestinal bleeding in a patient with Heyde syndrome with elevated factor VIII levels: A case report. Int J Crit Illn Inj Sci 2021; 11:253-256. [PMID: 35070916 PMCID: PMC8725811 DOI: 10.4103/ijciis.ijciis_151_20] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/26/2020] [Revised: 03/11/2021] [Accepted: 03/26/2021] [Indexed: 11/23/2022] Open
Abstract
Heyde syndrome is the association between gastrointestinal (GI) bleeding from intestinal angiodysplasia (IA) and aortic stenosis (AS). Although the course of disease progression that links AS and GI bleeding has not been determined, overlaps among AS, intestinal dysplasia, and acquired von Willebrand's syndrome is thought to result in GI bleeding. Proper repair of the aortic valve can result in significant improvement of GI bleeding and its recurrence. Herein, we are reporting this rare case, in which a patient with moderate AS on echocardiogram presents with recurrent GI bleeding from multiple IA in the setting of elevated factor VIII levels, to propose a theory that angiodysplasia could potentially develop due to intermittent, recurrent low-grade obstruction of submucosal veins at the level of the muscularis propria secondary to venous thrombosis related to elevated factor VIII levels.
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Affiliation(s)
- Omar Al-Radaideh
- Department of Medical Education, Saint Michael's Medical Center, New York Medical College, Newark, NJ, USA
| | - Iyad Farouji
- Department of Medical Education, Saint Michael's Medical Center, New York Medical College, Newark, NJ, USA
| | - Hossam Abed
- Department of Medical Education, Saint Michael's Medical Center, New York Medical College, Newark, NJ, USA
| | - Hamid Shaaban
- Department of Medical Education, Saint Michael's Medical Center, New York Medical College, Newark, NJ, USA.,Department of Hematology/Oncology, Saint Michael's Medical Center, New York Medical College, Newark, NJ, USA
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10
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Lourdusamy D, Mupparaju VK, Sharif NF, Ibebuogu UN. Aortic stenosis and Heyde’s syndrome: A comprehensive review. World J Clin Cases 2021; 9:7319-7329. [PMID: 34616798 PMCID: PMC8464459 DOI: 10.12998/wjcc.v9.i25.7319] [Citation(s) in RCA: 17] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/25/2021] [Revised: 07/07/2021] [Accepted: 07/26/2021] [Indexed: 02/06/2023] Open
Abstract
Heyde’s syndrome is an under reported systemic disease of gastrointestinal and cardiac manifestation in older adults. It is characterized by a triad of aortic stenosis, angiodysplasia with bleeding and acquired von Willebrand syndrome. It is characterized by proteolysis of high molecular weight multimers of von Willebrand Factor and loss of platelet mediated homeostasis. Heyde’s syndrome is a treatable condition in most cases, especially in the current era of evolution in interventional cardiology and gastroenterology. There are currently no established guidelines in the management of this condition due to paucity of high quality studies, which warrant future trials. High index of suspicion and increasing the awareness of the syndrome among the general practitioners and sub-specialists will improve the diagnostic potential of Heyde’s syndrome. Future studies may change the management aspect of Heyde's syndrome and pave a path for drawing specific guidelines and algorithms. The aim of our review article is to summarize the basic pathophysiology, diagnostics and management of Heyde’s syndrome with a special attention to Transcatheter aortic valve replacement.
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Affiliation(s)
| | | | - Navila Fahmida Sharif
- Internal Medicine, University of Tennessee Health Science Center, Memphis, TN 38103, United States
| | - Uzoma N Ibebuogu
- Cardiovascular Diseases, University of Tennessee Health Science Center, Memphis, TN 38163, United States
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11
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Belov DV, Garbuzenko DV, Lukin OP, Anufrieva SS. Heyde’s syndrome as a rare cause of gastrointestinal bleeding in aortic stenosis patients. КАРДИОВАСКУЛЯРНАЯ ТЕРАПИЯ И ПРОФИЛАКТИКА 2021. [DOI: 10.15829/1728-8800-2021-2503] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022] Open
Affiliation(s)
- D. V. Belov
- Federal Center for Cardiovascular Surgery; South Ural State Medical University
| | | | - O. P. Lukin
- Federal Center for Cardiovascular Surgery; South Ural State Medical University
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Mondal S, Hollander KN, Ibekwe SO, Williams B, Tanaka K. Heyde Syndrome-Pathophysiology and Perioperative Implications. J Cardiothorac Vasc Anesth 2020; 35:3331-3339. [PMID: 33132021 DOI: 10.1053/j.jvca.2020.10.003] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/05/2020] [Revised: 10/02/2020] [Accepted: 10/04/2020] [Indexed: 11/11/2022]
Abstract
Gastrointestinal (GI) bleeding in patients with calcific aortic valve stenosis (AVS), termed Heyde syndrome, was first described by Edward C. Heyde. The strong association between valvular replacement and the eradication of clinically significant GI bleeding confirmed an underlying pathophysiologic relationship. The rheologic stress created by AVS increases proteolysis of von Willebrand factor (VWF), resulting in loss of predominantly high-molecular-weight VWF (Hmw VWF). Angiodysplastic vessels present in patients with AVS, coupled with the lack of functioning Hmw VWF, increase the risk for GI bleeds. Aortic valve replacement, both surgical and transcatheter-based, is often a definitive treatment for GI bleeding, leading to recovery of Hmw VWF multimers. Perioperative management of patients involves monitoring their coagulation profiles with relevant laboratory tests and instituting appropriate management. Management can be directed in the following two ways: by improving internal release of VWF or by administration of external therapeutics containing VWF. It is important for perioperative physicians to obtain an understanding of the pathophysiology of this disease process and closely monitor the bleeding pattern so that targeted therapies can be initiated.
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Affiliation(s)
- Samhati Mondal
- Department of Anesthesiology, Cardiac Anesthesia Division, University of Maryland School of Medicine, Baltimore, MD.
| | - Kimberly N Hollander
- Department of Anesthesiology, Cardiac Anesthesia Division, University of Maryland School of Medicine, Baltimore, MD
| | - Stephanie O Ibekwe
- Department of Anesthesiology, Cardiovascular Division, BTGH, Baylor College of Medicine, Houston, TX
| | - Brittney Williams
- Department of Anesthesiology, Cardiac Anesthesia Division, University of Maryland School of Medicine, Baltimore, MD
| | - Kenichi Tanaka
- Department of Anesthesiology, Cardiac Anesthesia Division, University of Maryland School of Medicine, Baltimore, MD
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Abstract
Heyde syndrome is characterized by an association between gastrointestinal (GI) bleeding and calcific aortic stenosis (AS). Although the course of disease progression that links AS and GI bleeding has not been determined, overlaps among AS, intestinal dysplasia, and acquired von Willebrand's syndrome are thought to result in GI bleeding. Aortic valve repair in some patients has been reported to result in marked improvement or the complete resolution of signs and symptoms of Heyde syndrome. The prevalence of Heyde syndrome is higher among elderly persons than among other age groups, suggesting that a degenerative process may be a significant factor in the disease progression. This report describes a patient with Heyde syndrome, as well as a review of the current literature.
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Affiliation(s)
| | - Saul Vera
- Medicine, Dorrington Medical Associates, Houston, USA
| | - Munish Sharma
- Internal Medicine, Corpus Christi Medical Center, Corpus Christi, USA
| | - Joseph Varon
- Critical Care, University of Texas Health Science Center and United General Hospital, Houston, USA.,Internal Medicine: Critical Care, University of Texas Health Science Center and St Luke's Episcopal Hospital, Houston, USA
| | - Salim Surani
- Internal Medicine, Corpus Christi Medical Center, Corpus Christi, USA.,Internal Medicine, University of North Texas, Dallas, USA
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Tsuchiya S, Matsumoto Y, Doman T, Fujiya T, Sugisawa J, Suda A, Sato K, Ikeda S, Shindo T, Kikuchi Y, Hao K, Takahashi J, Hatta W, Koike T, Masamune A, Saiki Y, Horiuchi H, Shimokawa H. Disappearance of Angiodysplasia Following Transcatheter Aortic Valve Implantation in a Patient with Heyde's Syndrome: A Case Report and Review of the Literature. J Atheroscler Thromb 2019; 27:271-277. [PMID: 31378751 PMCID: PMC7113142 DOI: 10.5551/jat.49239] [Citation(s) in RCA: 14] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/16/2022] Open
Abstract
An 83-year-old woman with severe aortic stenosis was admitted to our hospital due to heart failure with refractory anemia requiring blood transfusions. She had repetitive bleeding episodes from endoscopically proven angiodysplasia in the stomach. Moreover, she repeatedly underwent endoscopic argon plasma coagulation for hemostasis. Importantly, she had a deficiency of the high-molecular-weight (HMW) multimers of von Willebrand factor (VWF), and she was diagnosed with Heyde's syndrome.After she underwent transcatheter aortic valve implantation (TAVI), aortic valve area and mean left ventricular aorta pressure gradient improved. Notably, endoscopy showed cessation of bleeding at 10 days after TAVI and the disappearance of angiodysplasia at 4 months after TAVI. Even at 2 years after TAVI, follow-up endoscopy showed remaining free of angiodysplasia in the stomach. She experienced no episodes of anemia since TAVI procedure. Additionally, analysis of HMW multimers demonstrated immediate and lasting recovery after TAVI.Recovery of HMW multimers of VWF with cessation of gastrointestinal bleeding following aortic valve replacement has been previously reported in a patient diagnosed with Heyde's syndrome. To the best our knowledge, this is the first case to demonstrate that angiodysplasia disappears after TAVI for a long term with endoscopic images in a patient with Heyde's syndrome. Here, we summarized case reports of patients with Heyde's syndrome that required aortic valve intervention. Cessation of gastrointestinal bleeding and anemia after aortic valve intervention for severe aortic stenosis may be attributed not only to recovery of HMW multimers of VWF but also to the disappearance of angiodysplasia.
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Affiliation(s)
- Satoshi Tsuchiya
- Department of Cardiovascular Medicine, Tohoku University Graduate School of Medicine
| | - Yasuharu Matsumoto
- Department of Cardiovascular Medicine, Tohoku University Graduate School of Medicine
| | - Tsuyoshi Doman
- Department of Molecular and Cellular Biology, Institute of Development, Aging, and Cancer, Tohoku University
| | - Taku Fujiya
- Department of Gastroenterology, Tohoku University Graduate School of Medicine
| | - Jun Sugisawa
- Department of Cardiovascular Medicine, Tohoku University Graduate School of Medicine
| | - Akira Suda
- Department of Cardiovascular Medicine, Tohoku University Graduate School of Medicine
| | - Koichi Sato
- Department of Cardiovascular Medicine, Tohoku University Graduate School of Medicine
| | - Shohei Ikeda
- Department of Cardiovascular Medicine, Tohoku University Graduate School of Medicine
| | - Tomohiko Shindo
- Department of Cardiovascular Medicine, Tohoku University Graduate School of Medicine
| | - Yoku Kikuchi
- Department of Cardiovascular Medicine, Tohoku University Graduate School of Medicine
| | - Kiyotaka Hao
- Department of Cardiovascular Medicine, Tohoku University Graduate School of Medicine
| | - Jun Takahashi
- Department of Cardiovascular Medicine, Tohoku University Graduate School of Medicine
| | - Waku Hatta
- Department of Gastroenterology, Tohoku University Graduate School of Medicine
| | - Tomoyuki Koike
- Department of Gastroenterology, Tohoku University Graduate School of Medicine
| | - Atsushi Masamune
- Department of Gastroenterology, Tohoku University Graduate School of Medicine
| | - Yoshikatsu Saiki
- Department of Cardiovascular Surgery, Tohoku University Graduate school of Medicine
| | - Hisanori Horiuchi
- Department of Molecular and Cellular Biology, Institute of Development, Aging, and Cancer, Tohoku University
| | - Hiroaki Shimokawa
- Department of Cardiovascular Medicine, Tohoku University Graduate School of Medicine
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