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Acquired von Willebrand syndrome in adult patients with congenital heart disease


Waldow, Hans Christian; Westhoff-Bleck, Mechthild; Widera, Christian; Templin, Christian; von Depka, Mario (2014). Acquired von Willebrand syndrome in adult patients with congenital heart disease. International Journal of Cardiology, 176(3):739-45.

Abstract

OBJECTIVES: Postoperative bleeding is common in patients with congenital heart disease (CHD). However, little is known about the role and prevalence of acquired von Willebrand syndrome (AVWS).
METHODS: We evaluated the prevalence of AVWS in relation to underlying cardiac defects, operative procedures and the presence of Eisenmenger syndrome. The prothrombin time, aPTT, platelet function analysis (PFA-100), von Willebrand factor antigen (VWF:Ag), VWF activity, VWF collagen binding activity (VWF:CB), factor VIII activity and multimeric analysis were measured in addition to tests evaluating heart, liver and kidney functions.
RESULTS: A total of 221 patients were screened and 192 patients were included in the study. The overall prevalence of AVWS was 20.8%. AVWS was identified across all of the cardiac defects, with the highest prevalence in the defects of great complexity (38.6% compared to 9.4% in patients with CHD of simple/moderate complexity, p<0.001), Eisenmenger syndrome (p<0.001) and more severe heart failure symptoms (NYHA III/IV vs. NYHA I, p<0.001; NYHA III/IV vs. NYHA II, p=0.044). A combination of multimeric analysis, VWF:CB to VWF:Ag ratio (sensitivity: 77.5%, specificity: 93.3%) and PFA-100 (PFA Col/Epi sens.: 77%, spec.: 52%; PFA Col/ADP sens.: 75%, spec.: 74.3%) were used to detect AVWS.
CONCLUSIONS: This study demonstrated that AVWS occurred in patients with various congenital cardiac defects, but the highest prevalence occurred in the patients with complex CHD and Eisenmenger syndrome. We, therefore, suggest preoperative screening for AVWS in all of the patients with CHD, particularly in the patients with CHD of greater complexity and suffering from Eisenmenger syndrome.

Abstract

OBJECTIVES: Postoperative bleeding is common in patients with congenital heart disease (CHD). However, little is known about the role and prevalence of acquired von Willebrand syndrome (AVWS).
METHODS: We evaluated the prevalence of AVWS in relation to underlying cardiac defects, operative procedures and the presence of Eisenmenger syndrome. The prothrombin time, aPTT, platelet function analysis (PFA-100), von Willebrand factor antigen (VWF:Ag), VWF activity, VWF collagen binding activity (VWF:CB), factor VIII activity and multimeric analysis were measured in addition to tests evaluating heart, liver and kidney functions.
RESULTS: A total of 221 patients were screened and 192 patients were included in the study. The overall prevalence of AVWS was 20.8%. AVWS was identified across all of the cardiac defects, with the highest prevalence in the defects of great complexity (38.6% compared to 9.4% in patients with CHD of simple/moderate complexity, p<0.001), Eisenmenger syndrome (p<0.001) and more severe heart failure symptoms (NYHA III/IV vs. NYHA I, p<0.001; NYHA III/IV vs. NYHA II, p=0.044). A combination of multimeric analysis, VWF:CB to VWF:Ag ratio (sensitivity: 77.5%, specificity: 93.3%) and PFA-100 (PFA Col/Epi sens.: 77%, spec.: 52%; PFA Col/ADP sens.: 75%, spec.: 74.3%) were used to detect AVWS.
CONCLUSIONS: This study demonstrated that AVWS occurred in patients with various congenital cardiac defects, but the highest prevalence occurred in the patients with complex CHD and Eisenmenger syndrome. We, therefore, suggest preoperative screening for AVWS in all of the patients with CHD, particularly in the patients with CHD of greater complexity and suffering from Eisenmenger syndrome.

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4 citations in Web of Science®
5 citations in Scopus®
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Additional indexing

Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Clinic for Cardiology
Dewey Decimal Classification:610 Medicine & health
Language:English
Date:20 October 2014
Deposited On:11 Feb 2015 09:39
Last Modified:05 Apr 2016 19:04
Publisher:Elsevier
ISSN:0167-5273
Publisher DOI:https://doi.org/10.1016/j.ijcard.2014.07.104
PubMed ID:25139318

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