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    Poster Networking

    Hemophilia and Rare Bleeding Disorders

    Poster Networking Session

    PB1286 - Cardiac surgery in a patient with severe haemophilia A receiving emicizumab prophylaxis - A case report

    Tuesday, June 27, 2023
    18:30 – 19:30 ET
    Room: Exhibition

      Presenting Author(s)

    • Bharath Ram Sreedhar, DNB (he/him/his)

      Senior Clinical Fellow
      Katharine Dormandy Haemophilia and Thrombosis Centre, Royal Free London NHS Foundation Trust. London, United Kingdom
      London, England, United Kingdom

      • Co-Author(s)

    • CM

      Carolyn Maharaj

      Haematology Registrar
      Katharine Dormandy Haemophilia and Thrombosis Centre, Royal Free London NHS Foundation Trust. London, United Kingdom
      London, England, United Kingdom

    • LM

      Laura JB Mawer, BA MBBS BChir MRCP PGCert MedEd

      Haematology Doctor (Registrar)
      Katharine Dormandy Haemophilia and Thrombosis Centre, Royal Free London NHS Foundation Trust. London, United Kingdom
      London, England, United Kingdom

    • Paul Batty, MBBS, PhD (he/him/his)

      Associate Professor
      University College London
      London, United Kingdom

    • AR

      Anne Riddell

      Laboratory Head of Department (Special Coagulation, Haemophilia) and HSL Scientific Lead Coagulation
      Katharine Dormandy Haemophilia and Thrombosis Centre, Royal Free Hospital, London, UK
      London, England, United Kingdom

    • Molly Musarara, RN, BSC(Hons)

      Haemophilia Clinical Nurse Specialist
      Katharine Dormandy Haemophilia and Thrombosis Centre, Royal Free London NHS Foundation Trust, London, UK
      London, England, United Kingdom

    • EA

      Elsa Aradom

      Lead Nurse, Haemophilia
      Katharine Dormandy Haemophilia and Thrombosis Centre, Royal Free London NHS Foundation Trust, London, UK
      London, England, United Kingdom

    • FD

      Fabio De Robertis

      Consultant Cardiac and Transplant Surgeon
      Royal Brompton and Harefield Hospitals, London, United Kingdom
      London, England, United Kingdom

    • SL

      Silvia Lovato

      Consultant Haematologist
      Royal Brompton and Harefield Hospitals, London, United Kingdom
      London, England, United Kingdom

    • AV

      Alessandra Verzelloni Sef

      Consultant in Anaesthesia and Critical Care
      Royal Brompton and Harefield Hospitals, London, United Kingdom
      London, England, United Kingdom

    • GC

      Giuditta Coppola

      Specialist Registrar, Cardiothoracic surgery
      Royal Brompton and Harefield Hospitals, London, United Kingdom
      London, England, United Kingdom

    • PC

      Pratima Chowdary, MD

      Consultant Haematologist
      Katharine Dormandy Haemophilia and Thrombosis Centre, Royal Free London NHS Foundation Trust, London, United Kingdom
      London, England, United Kingdom

    Background: Cardiac surgery in haemophilia presents challenges due to heparin utilization on cardiopulmonary bypass, further complicated by the use of emicizumab. Although ex-vivo spiking studies report no effect of emicizumab on the activated clotting time (ACT), there is limited real-world experience in peri-operative monitoring of heparin with ACT.

    Aims: To describe the complexity of management of cardiac surgery in a patient with severe haemophilia A on emicizumab.

    Methods: Peri-operative planning was performed via virtual multi-disciplinary team meetings, due to surgical unit being located 20 miles away from the haemophilia centre. Point-of-care monitoring was performed using the ACT. Plasma samples were couriered to haemophilia centre. Factor VIII activity (FVIII:C) and emicizumab levels were measured using a bovine chromogenic assay and modified one-stage assay respectively.

    Results: A patient in early 50s with severe haemophilia A was diagnosed with severe mitral regurgitation requiring valve repair after hospitalization with atrial fibrillation and cardiac failure. He had been receiving emicizumab (105mg/week) for two years. The last dose of Emicizumab was 12 days pre-operatively (concentration: 36.8mcg/ml). Surgery was managed with standard half-life (SHL) FVIII concentrate bolus of 85IU/kg pre-operatively (post level 231IU/dl). An additional dose of 30IU/kg was administered while on bypass circuit. Intra-operative haemostasis was excellent (end-of-surgery FVIII:C-164IU/dL). There was a need for additional heparin boluses during surgery. Continuous infusion was used post-operatively to maintain FVIII:C >100IU/dl and continued till day 20. High blood output was noted in the drains 6 hrs post-surgery requiring relook without intervention. A left atrial appendage thrombus was diagnosed on day 14, requiring anticoagulation (enoxaparin followed by edoxaban), with bolus SHL FVIII twice a day, aiming for a trough level of 50%.

    Conclusion(s): Cardiac surgery in patients with haemophilia on emicizumab requires a multi-disciplinary approach with a need for more understanding on the impact of emicizumab on ACT monitoring in the context of cardiac surgery.