Acquired von Willebrand syndrome (AVWS) type 2, characterized by decreased high molecular weight multimers, is common in children with severe pulmonary hypertension (PH)
Background and objectivesEmerging evidence suggests that increased degradation of von Willebrand factor and decrease in high molecular weight multimers occurs in patients with pulmonary hypertension (PH). However, the link between acquired von Willebrand Syndrome (AVWS) type 2 and PH remains poorly...
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2022-11-01
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| Series: | Frontiers in Pediatrics |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fped.2022.1012738/full |
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| author | Ivonne Wieland Franziska Diekmann Julia Carlens Laura Hinze Katharina Lambeck Thomas Jack Georg Hansmann |
| author_facet | Ivonne Wieland Franziska Diekmann Julia Carlens Laura Hinze Katharina Lambeck Thomas Jack Georg Hansmann |
| author_sort | Ivonne Wieland |
| collection | DOAJ |
| description | Background and objectivesEmerging evidence suggests that increased degradation of von Willebrand factor and decrease in high molecular weight multimers occurs in patients with pulmonary hypertension (PH). However, the link between acquired von Willebrand Syndrome (AVWS) type 2 and PH remains poorly understood.Material and methodsWe retrospectively evaluated the charts of 20 children with PH who underwent bilateral lung transplantation (LuTx) between 2013 and 2022. Von Willebrand variables were determined in 14 of these patients; 11 patients had complete diagnostics including multimer analysis.ResultsWe confirmed AVWS in 82% of the children studied (9 of 11 patients by multimer analysis). The two remaining patients had suspected AVWS type 2 because of a VWF:Ac/VWF:Ag ratio of <0.7. Platelet dysfunction or suspicion of VWD type 1 were found in two separate patients. All but one of the 14 children with severe PH had a coagulation disorder. Most patients (9 proven, 2 suspected) had AVWS type 2. Notably, 3 of 5 patients (60%) with normal VWF:Ac/VWF:Ag ratio >0.7 had abnormal VWF multimers, indicating AVWS type 2. Hemostatic complications were observed in 4 of 12 (33%) patients with VWS and 3 of 6 (50%) patients without diagnostics and therapy.ConclusionFor children with moderate to severe PH, we recommend systematic analysis of von Willebrand variables, including multimer analysis, PFA-100 and platelet function testing. Awareness of the diagnosis “AVWS” and adequate therapy may help to prevent these patients from bleeding complications in case of surgical interventions or trauma. |
| first_indexed | 2024-04-12T08:58:08Z |
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| id | doaj.art-3b14480e964d477f958b50c965cf65ef |
| institution | Directory Open Access Journal |
| issn | 2296-2360 |
| language | English |
| last_indexed | 2024-04-12T08:58:08Z |
| publishDate | 2022-11-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Pediatrics |
| spelling | doaj.art-3b14480e964d477f958b50c965cf65ef2022-12-22T03:39:19ZengFrontiers Media S.A.Frontiers in Pediatrics2296-23602022-11-011010.3389/fped.2022.10127381012738Acquired von Willebrand syndrome (AVWS) type 2, characterized by decreased high molecular weight multimers, is common in children with severe pulmonary hypertension (PH)Ivonne Wieland0Franziska Diekmann1Julia Carlens2Laura Hinze3Katharina Lambeck4Thomas Jack5Georg Hansmann6Department of Pediatric Hematology and Oncology, Hannover Medical School, Hannover, GermanyDepartment of Pediatric Cardiology and Critical Care, Hannover Medical School, Hannover, GermanyDepartment of Pediatric Pulmonology, Allergology, and Neonatology, Hannover Medical School, Hannover, GermanyDepartment of Pediatric Hematology and Oncology, Hannover Medical School, Hannover, GermanyDepartment of Pediatric Hematology and Oncology, Hannover Medical School, Hannover, GermanyDepartment of Pediatric Cardiology and Critical Care, Hannover Medical School, Hannover, GermanyDepartment of Pediatric Cardiology and Critical Care, Hannover Medical School, Hannover, GermanyBackground and objectivesEmerging evidence suggests that increased degradation of von Willebrand factor and decrease in high molecular weight multimers occurs in patients with pulmonary hypertension (PH). However, the link between acquired von Willebrand Syndrome (AVWS) type 2 and PH remains poorly understood.Material and methodsWe retrospectively evaluated the charts of 20 children with PH who underwent bilateral lung transplantation (LuTx) between 2013 and 2022. Von Willebrand variables were determined in 14 of these patients; 11 patients had complete diagnostics including multimer analysis.ResultsWe confirmed AVWS in 82% of the children studied (9 of 11 patients by multimer analysis). The two remaining patients had suspected AVWS type 2 because of a VWF:Ac/VWF:Ag ratio of <0.7. Platelet dysfunction or suspicion of VWD type 1 were found in two separate patients. All but one of the 14 children with severe PH had a coagulation disorder. Most patients (9 proven, 2 suspected) had AVWS type 2. Notably, 3 of 5 patients (60%) with normal VWF:Ac/VWF:Ag ratio >0.7 had abnormal VWF multimers, indicating AVWS type 2. Hemostatic complications were observed in 4 of 12 (33%) patients with VWS and 3 of 6 (50%) patients without diagnostics and therapy.ConclusionFor children with moderate to severe PH, we recommend systematic analysis of von Willebrand variables, including multimer analysis, PFA-100 and platelet function testing. Awareness of the diagnosis “AVWS” and adequate therapy may help to prevent these patients from bleeding complications in case of surgical interventions or trauma.https://www.frontiersin.org/articles/10.3389/fped.2022.1012738/fullacquired von willebrand syndrome (AVWS)pulmonary hypertensionbleeding riskhigh molecular weight multimerschildren |
| spellingShingle | Ivonne Wieland Franziska Diekmann Julia Carlens Laura Hinze Katharina Lambeck Thomas Jack Georg Hansmann Acquired von Willebrand syndrome (AVWS) type 2, characterized by decreased high molecular weight multimers, is common in children with severe pulmonary hypertension (PH) Frontiers in Pediatrics acquired von willebrand syndrome (AVWS) pulmonary hypertension bleeding risk high molecular weight multimers children |
| title | Acquired von Willebrand syndrome (AVWS) type 2, characterized by decreased high molecular weight multimers, is common in children with severe pulmonary hypertension (PH) |
| title_full | Acquired von Willebrand syndrome (AVWS) type 2, characterized by decreased high molecular weight multimers, is common in children with severe pulmonary hypertension (PH) |
| title_fullStr | Acquired von Willebrand syndrome (AVWS) type 2, characterized by decreased high molecular weight multimers, is common in children with severe pulmonary hypertension (PH) |
| title_full_unstemmed | Acquired von Willebrand syndrome (AVWS) type 2, characterized by decreased high molecular weight multimers, is common in children with severe pulmonary hypertension (PH) |
| title_short | Acquired von Willebrand syndrome (AVWS) type 2, characterized by decreased high molecular weight multimers, is common in children with severe pulmonary hypertension (PH) |
| title_sort | acquired von willebrand syndrome avws type 2 characterized by decreased high molecular weight multimers is common in children with severe pulmonary hypertension ph |
| topic | acquired von willebrand syndrome (AVWS) pulmonary hypertension bleeding risk high molecular weight multimers children |
| url | https://www.frontiersin.org/articles/10.3389/fped.2022.1012738/full |
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