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.
SudhakarMRikhiRLoganathanSK, et al.Autoimmunity in Wiskott-Aldrich syndrome: updated perspectives. Appl Clin Genet2021; 20(14): 363–388. https://doi.org/10.2147/TACG.S213920
4.
BoldtJKnotheCZickmannB, et al.Platelet function in cardiac surgery: influence of temperature and aprotinin. Ann Thorac Surg1993; 55(3): 652–658. https://doi.org/10.1016/0003-4975(93)90269-n
BernabeuEJosaMNomdedeuB, et al.One-step surgical approach of a thoracic aortic aneurysm in Wiskott-Aldrich syndrome. Ann Thorac Surg2007; 83(4): 1537–1538. https://doi.org/10.1016/j.athoracsur.2006.10.005
7.
CentofantiPBarberoCD'AgataF, et al.Neurologic and cognitive outcomes after aortic arch operation with hypothermic circulatory arrest. Surgery2016; 160(3): 796–804. https://doi.org/10.1016/j.surg.2016.02.008
8.
MahmoodSBilalHZamanM, et al.Is a fully heparin-bonded cardiopulmonary bypass circuit superior to a standard cardiopulmonary bypass circuit. Interact Cardiovasc Thorac Surg2012; 14(4): 406–414. https://doi.org/10.1093/icvts/ivr124
9.
WeberNWendelHPZiemerG. Quality assessment of heparin coatings by their binding capacities of coagulation and complement enzymes. J Biomater Appl2000; 15(1): 8–22. https://doi.org/10.1106/DUYU-CEL6-529C-WUL9
10.
OvrumETangenGOysteseR, et al.Comparison of two heparin-coated extracorporeal circuits with reduced systemic anticoagulation in routine coronary artery bypass operations. J Thorac Cardiovasc Surg2001; 121(2): 324–330. https://doi.org/10.1067/mtc.2001.111205
11.
SavageBSaldívarERuggeriZM. Initiation of platelet adhesion by arrest onto fibrinogen or translocation on von Willebrand factor. Cell1996; 84(2): 289–297. https://doi.org/10.1016/s0092-8674(00)80983-6
12.
KeelingDTaitRCWatsonH, et al.Peri-operative management of anticoagulation and antiplatelet therapy. Br J Haematol2016; 175: 602–613. https://doi.org/10.1111/bjh.14344
13.
NarayanPAlwairHBryanAJ. Surgical resection of sequential thoracic aortic aneurysms in Wiskott-Aldrich syndrome. Interact Cardiovasc Thorac Surg2004; 3(2): 346–348. https://doi.org/10.1016/j.icvts.2004.02.001
14.
Van SonJAO’MarcaighASEdwardsWD, et al.Successful resection of thoracic aortic aneurysms in Wiskott-Aldrich syndrome. Ann Thorac Surg1995; 60(3): 685–687. https://doi.org/10.1016/0003-4975(95)00171-G
