Acquired von Willebrand syndrome in children with aortic and pulmonary stenosis

Abstract

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.

Von Willebrand Factor as a Novel Player in Valvular Heart Disease: From Bench to Valve Replacement

“…Few studies in literature, mainly case reports and series, indicate a possible correlation between AvWS and congenital 56,57 or acquired 58 pulmonary stenosis (PS). Pulmonary stenosis is one of the features of patients with Noonan syndrome (NS).…”

“…In children with NS and PS, deficiency of HMW-vWF multimers and bleeding diathesis have been described, suggesting a diagnosis of AvWS. 56,57 Since most of these children require either interventional cardiac catheterization or open-heart surgery, this finding may be relevant in the management of hemorrhagic complications. In the literature, no data on vWF function in tricuspid valve disease have been published.…”

Acquired von Willebrand Syndrome in Children

“…Following interventional PDA occlusion, the VWF HMW multimers normalized shortly after the intervention in all patients, confirming the acquired nature of the disorder. 9 In addition, the frequency and relationship of AVWS in children with aortic and pulmonary stenosis were investigated by Binnetoğlu et al 10 AVWS was found to be associated with stenotic obstructive cardiac diseases. Therefore, laboratory analyses should comprise comprehensive analysis of VWF parameters in these patients besides whole blood count, prothrombin time, and activated partial thromboplastin time.…”

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

“…In a study of children with either congenital pulmonic or aortic stenosis, there was a history of bleeding in 9% and 18%, respectively. Seven of the 60 children (12%) had laboratory findings consistent with a diagnosis of AVWS, and two of these (28%) had a history of bleeding [39].…”