Acquired von Willebrand Syndrome in congenital heart disease: does it promote an increased bleeding risk?

Onimoe, Grace; Grooms, Linda; Perdue, Kami; Ruymann, Frederick B.

doi:10.1111/j.1365-2141.2011.08732.x

Cited by 18 publications

(12 citation statements)

References 9 publications

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“…Some authors have observed very low rates of bleeding in patients with AVWS secondary to CPD (Casonato et al , ; Loeffelbein et al , ). Conversely, some authors have reported increased rates of post‐operative and intracranial bleeding in the setting of congenital heart disease and/or use of ventricular assist devices (Onimoe et al , ; Jones et al , ). In our cohort, most patients exhibited only mild bleeding symptoms; the one patient that underwent a double‐lung transplantation did sustain peri‐operative haemorrhage despite having received a VWF concentrate.…”

Section: Discussionmentioning

confidence: 99%

Idiopathic pulmonary arterial hypertension – a unrecognized cause of high‐shear high‐flow haemostatic defects (otherwise referred to as acquired von Willebrand syndrome) in children

et al. 2018

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Acquired von Willebrand syndrome (AVWS) is reported in high-flow high-shear congenital cardiac disorders. We hypothesized that the narrowed pulmonary vasculature in idiopathic pulmonary arterial hypertension (IPAH) may induce AVWS. We conducted a cross-sectional evaluation of children with IPAH. Patients with bleeding symptoms and/or laboratory abnormalities (thrombocytopenia, anomalies in coagulation screening tests) were tested in-depth for haemostatic defects. Fourteen children were followed with IPAH of which 8 were eligible. Four children exhibited abnormal bleeding scores (International Society on Thrombosis and Haemostasis Bleeding Assessment Tool: 3-5). All 8 patients showed very prolonged platelet function analyser (PFA)-100 closure times. Six children demonstrated either mild thrombocytopenia or low-normal von Willebrand factor (VWF) antigen (VWF:Ag) or VWF activity [mean (range), in iu/dl: VWF:Ag: 70 (61-91); VWF activity: 57 (34-70)]. Average VWF collagen binding capacity (VWF:CB) was 64 iu/dl (range: 53-123 iu/dl), with low-normal VWF activity/VWF:Ag or VWF:CB/VWF:Ag ratios occurring in five patients. All children had normal multimers distribution patterns. One patient underwent a lung transplantation, with normalization of haemostatic abnormalities post-surgery. Overall, 8 out of 14 children with IPAH had mild to moderate bleeding symptoms and/or laboratory abnormalities in keeping with AVWS. Normalization of the haemostatic defects following lung transplantation and lack of family history of bleeding attests to the acquired nature of their defects.

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Section: Discussionmentioning

confidence: 99%

Idiopathic pulmonary arterial hypertension – a unrecognized cause of high‐shear high‐flow haemostatic defects (otherwise referred to as acquired von Willebrand syndrome) in children

et al. 2018

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“…cause high shear stress. Acquired von Willebrand syndrome IIA has been reported in patients with hypertrophic obstructive cardiomyopathy 14) , congenital structural cardiac diseases 15) , pulmonary hypertension 16) , and mitral stenosis 17) . Furthermore, mechanical circulatory support has drastically advanced and contributes much to the treatment of severe heart failure and it is inevitable that high shear stress is generated inside the pumps used in the devices.…”

Section: Treatment and Prognosis Of Patients With Heyde's Syndromementioning

confidence: 99%

Unexpectedly High Prevalence of Acquired von Willebrand Syndrome in Patients with Severe Aortic Stenosis as Evaluated with a Novel Large Multimer Index

Tamura

Horiuchi

Imai

et al. 2015

J Atheroscler Thromb

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Aim:Severe gastrointestinal bleeding sometimes occurs in patients with aortic stenosis (AS), known as Heyde's syndrome. This syndrome is thought to be caused by acquired von Willebrand syndrome and is characterized by reduced large von Willebrand factor (vWF) multimers. However, the relationship between the severity of AS and loss of large vWF multimers is unclear. Methods: We examined 31 consecutive patients with severe AS. Quantitative evaluation for loss of large vWF multimers was performed using the conventional large vWF ratio and novel large vWF multimer index. This novel index was defined as the ratio of large multimers of patients to those of controls. Results: Loss of large vWF multimers, defined as the large vWF multimer index 80%, was detected in 21 patients (67.7%). The large vWF multimer ratio and the large vWF multimer index were inversely correlated with the peak aortic gradient (R 0.58, p 0.0007, and R 0.64, p 0.0001, respectively). Anemia defined as hemoglobin 9.0 g/dl was observed in 12 patients (38.7%), who were regarded as Heyde's syndrome. Aortic valve replacement was performed in 7 of these patients, resulting in the improvement of anemia in all patients from a hemoglobin concentration of 7.5 1.0 g/dl preoperatively to 12.4 1.3 g/dl postoperatively (p 0.0001). Conclusions: Acquired von Willebrand syndrome may be a differential diagnosis in patients with AS with anemia. The prevalence of AS-associated acquired von Willebrand syndrome is higher than anticipated.

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“…Ventricular Septal Defects-There have been reports of AVWS in patients with congenital ventricular septal defects with resolution following corrective surgery [47].…”

Section: Prevalence Of Avws In Cardiovascular Diseasementioning

confidence: 99%

Acquired Von Willebrand Syndrome (AVWS) in cardiovascular disease: a state of the art review for clinicians

Mehta

Athar

Girgis

et al. 2019

J Thromb Thrombolysis

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Von Willebrand Factor (vWF) is a large glycoprotein with a broad range of physiological and pathological functions in health and disease. While vWF is critical for normal hemostasis, vascular integrity and repair, quantitative and qualitative abnormalities in the molecule can predispose to serious bleeding and thrombosis. The heritable form of von Willebrand Disease was first described nearly a century ago, but more recently, recognition of an acquired condition known as acquired von Willebrand Syndrome (AVWF) has emerged in persons with hematological, endocrine and cardiovascular diseases, disorders and conditions. An in-depth understanding of the causes, diagnostic approach and management of AVWS is important for practicing clinicians.

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Acquired von Willebrand Syndrome in congenital heart disease: does it promote an increased bleeding risk?

Abstract: Keywords: B cell activating factor, ELISA, thrombotic thrombocytopenic purpura, a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13, rituximab.

Cited by 18 publications

References 9 publications

Idiopathic pulmonary arterial hypertension – a unrecognized cause of high‐shear high‐flow haemostatic defects (otherwise referred to as acquired von Willebrand syndrome) in children

Idiopathic pulmonary arterial hypertension – a unrecognized cause of high‐shear high‐flow haemostatic defects (otherwise referred to as acquired von Willebrand syndrome) in children

Unexpectedly High Prevalence of Acquired von Willebrand Syndrome in Patients with Severe Aortic Stenosis as Evaluated with a Novel Large Multimer Index

Acquired Von Willebrand Syndrome (AVWS) in cardiovascular disease: a state of the art review for clinicians

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