Redo ascending aorta and total arch replacement in patient with Wiskott-Aldrich syndrome: A case report

Wiskott-Aldrich syndrome (WAS) is an X-linked genetic disorder characterised by eczema, immunodeficiency, and thrombocytopenia. Cardiopulmonary bypass causes a reduction in platelet numbers and function leading to an increased risk of bleeding. We report a case of a 33 year old male with WAS who underwent a redo ascending aorta and total aortic arch replacement as part of a two stage surgery for a Type B aortic dissection. The report discusses the considerations given to cardiopulmonary bypass in order to mitigate platelet number reduction and the degree of systemic inflammatory response. Post operative viscoelastometry indicated a minor hypocoagulopathy with minimal post-operative bleeding.