title: Successful aortic aneurysm repair in a woman with severe von Willebrand (type 3) disease.
abstract: von Willebrand disease type 3 (VWD3) is a rare but the most severe form of von Willebrand disease; it is due to almost complete lack of von Willebrand factor activity (VWF:RCo). It is inherited as autosomal recessive trait; whilst heterozygote carriers have mild, or no symptoms, patients with VWD3 show severe bleeding symptoms. In the laboratory, this is characterised by undetectable VWF:Ag, VWF:RCo, and reduced levels of factor VIII < 0.02 IU/dL. The bleeding is managed with von Willebrand/FVIII factor concentrate replacement therapy. In this rare but challenging case we report on the successful excision and repair of an ascending aortic aneurysm following adequate VWF/FVIII factor concentrate replacement using Haemate-P.
authors.affiliation: Department of Haematology, Imperial College Healthcare NHS Trust, London W2 1NY, UK.
authors.forename: Victoria
authors.initials: V
authors.lastname: Campbell
authors.affiliation: Department of Haematology, Imperial College Healthcare NHS Trust, London W2 1NY, UK.
authors.forename: Kevin
authors.initials: K
authors.lastname: Marriott
authors.affiliation: Department of Cardiothoracic Surgery, Imperial College Healthcare NHS Trust, London W2 1NY, UK.
authors.forename: Rex
authors.initials: R
authors.lastname: Stanbridge
authors.affiliation: Department of Haematology, Imperial College Healthcare NHS Trust, London W2 1NY, UK.
authors.forename: Abdul
authors.initials: A
authors.lastname: Shlebak