Acquired von Willebrand syndrome in children with patent ductus arteriosus

Rauch, Ralf; Budde, Ulrich; Koch, Andreas; Girisch, M.; Hofbeck, Michael

doi:10.1136/heart.88.1.87

Cited by 33 publications

(27 citation statements)

References 5 publications

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“…It is also hypothesized that subtle impairments in longitudinal systolic function may be missed due to the traditional focus on radial myocardial contraction and more sensitive techniques, such as strain and myocardial tissue Doppler velocity, may better detect early systolic abnormalities in patients with diabetes. 445,446 Taken together, these data suggest a spectrum of diabetic cardiomyopathy. 432 …”

Section: Diabetic Cardiomyopathy- Clinical Phenotypementioning

confidence: 69%

Clinical Update: Cardiovascular Disease in Diabetes Mellitus

et al. 2016

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Cardiovascular disease remains the principal cause of death and disability among patients with diabetes mellitus. Diabetes exacerbates mechanisms underlying atherosclerosis and heart failure. Unfortunately, these mechanisms are not adequately modulated by therapeutic strategies focusing solely on optimal glycemic control with currently available drugs or approaches. In the setting of multi-factorial risk reduction with statins and other lipid lowering agents, anti-hypertensive therapies, and anti-hyperglycemic treatment strategies, cardiovascular complication rates are falling, yet remain higher for patients with diabetes than for those without. This review considers the mechanisms, history, controversies, new pharmacologic agents, and recent evidence for current guidelines for cardiovascular management in the patient with diabetes mellitus to support evidence-based care in the patient with diabetes and heart disease outside of the acute care setting.

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Section: Diabetic Cardiomyopathy- Clinical Phenotypementioning

confidence: 69%

Clinical Update: Cardiovascular Disease in Diabetes Mellitus

et al. 2016

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“…Surgical repair of the ventricular defect was followed by normalization of the VWF multimeric pattern. In a systematic investigation, Rauch et al [20] observed a type 2-like aVWD in 4 of 12 children with persistent patent ductus arteriosis (PDA) [20]. After interventional PDA occlusion, the VWF multimers had normalized in all 4 with pathologic VWF, confirming the acquired nature of aVWD in PDA.…”

Section: Classification Of Avwdmentioning

confidence: 82%

Causes, Etiology and Diagnosis of Acquired von Willebrand Disease: A Prospective Diagnostic Workup to Establish the Most Effective Therapeutic Strategies

Sucker¹,

Michiels

Zotz³

2009

Acta Haematol

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Acquired von Willebrand disease (aVWD) occurs in association with a variety of underlying disorders, most frequently in lymphoproliferative and myeloproliferative disorders, other malignancies, and cardiovascular disease. aVWD is a complex and heterogeneous defect with a multifactorial etiology and the pathophysiologic mechanisms remain unclear in many cases. Assays for anti-factor VIII (FVIII)/von Willebrand factor (VWF) activities often yield negative results although antibodies may be present in autoimmune disease and some lymphoproliferative disorders. Functional assays of VWF in patients’ plasma and particularly in heart valve disease, VWF multimer analysis are important for aVWD diagnosis. In patients with normal partial thromboplastin times and normal VWF activity, the diagnosis of aVWD is based on clinical suspicion and a careful bleeding history, which should prompt the clinician to initiate further laboratory investigations. Management of bleeding in aVWD relies mainly on desmopressin, FVIII/VWF concentrates and high-dose intravenous immunoglobulin. The half-life of VWF may be very short, and in bleeding episodes high doses of FVIII/VWF concentrates at short intervals may be necessary even when high-dose intravenous immunoglobulin was applied before. Since the optimal treatment strategy has not yet been defined for aVWD of different etiology, controlled multicenter trials aiming at the development of standardized treatment protocols are urgently needed.

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“…Although Froom et al19 reported a relationship between frequent epistaxis episodes and low VWF:Ag, Rauch et al15 did not find any association between bleeding episodes and the von Willebrand deficiency in patients with patent ductus arteriosus. We also could not find any relationship between low VWF:Ag levels and bleeding.…”

Section: Discussionmentioning

confidence: 95%

Acquired von Willebrand syndrome in children with aortic and pulmonary stenosis

Binnetoğlu

Babaoğlu

Filiz

et al. 2016

CVJA

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SummaryIntroduction:This prospective study was planned to investigate the frequency and relationship of acquired von Willebrand syndrome (AVWS) with aortic and pulmonary stenosis in patients.Methods:A total of 84 children, ranging from two to 18 years of age, were enrolled in this study. Of these, 28 had isolated aortic stenosis, 32 had isolated pulmonary stenosis and 24 were healthy. Children with aortic and pulmonary stenosis associated with other congenital heart diseases were excluded. Children with hypothyroidism, renal or liver disease, malignancy or autoimmune disease were also excluded. Wholeblood count, blood group, factor VIII level, prothrombin time (PT), activated partial thromboplastin time (aPTT), von Willebrand factor antigen (VWF:Ag), ristocetin co-factor (VWF:RCo), and bleeding time using a platelet-function analyser (PFA-100) were performed in all patients. All of the children in the study underwent a detailed physical examination and echocardiographic evaluation.Results:A history of bleeding was positive in 18% of the aortic stenosis group, 9% of the pulmonary stenosis group, and 4% of the control group. Seven of 60 (12%) patients had laboratory findings that implied a diagnosis of AVWS, and two of these (28%) had a history of bleeding. The frequency of AVWS was 14% in patients with aortic stenosis and 9% in those with pulmonary stenosis.Conclusion:AVWS is not rare in stenotic obstructive cardiac diseases. A detailed history of bleeding should be taken from patients with valvular disease. Even if the history is negative, whole blood count, PT and aPTT should be performed. If necessary, PFA-100 closure time and further tests should be planned for the diagnosis of AVWS.

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Acquired von Willebrand syndrome in children with patent ductus arteriosus

Cited by 33 publications

References 5 publications

Clinical Update: Cardiovascular Disease in Diabetes Mellitus

Clinical Update: Cardiovascular Disease in Diabetes Mellitus

Causes, Etiology and Diagnosis of Acquired von Willebrand Disease: A Prospective Diagnostic Workup to Establish the Most Effective Therapeutic Strategies

Acquired von Willebrand syndrome in children with aortic and pulmonary stenosis

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