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Left atrial appendage occlusion in haemophilia patients with atrial fibrillation

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Journal of Interventional Cardiac Electrophysiology Aims and scope Submit manuscript

Abstract

Purpose

Advanced targeted therapy has resulted in increasing life expectancy and incidence of age-related cardiovascular diseases like atrial fibrillation in patients with haemophilia. Oral anticoagulation constitutes a significant dilemma in this patient category as the risks of stroke and bleeding are difficult to balance. We sought to demonstrate the feasibility of left atrial appendage occlusion (LAAO) in patients with haemophilia and atrial fibrillation.

Methods

All patients with haemophilia treated with LAAO at Aarhus University Hospital, Denmark, were identified from a local prospective database comprising all consecutive LAAO procedures from 2010 up to November 2020. Based on review of the medical records, a retrospective descriptive analysis was performed.

Results

Seven patients with haemophilia A and atrial fibrillation underwent LAAO after multidisciplinary conference. Peri-procedural coagulation management was guided by factor VIII activity and treated with repeated bolus administrations of recombinant factor VIII targeting an activity of 100%. The implantation was successful in all patients with only minor bleeding complications post-procedurally. Based on these experiences, a suggested regime has been formulated.

Conclusions

LAAO is feasible in haemophilia patients with concurrent atrial fibrillation. However, special care including intravenous substitution with coagulation factors must be given in the periprocedural management.

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Data availability

Data supporting the findings of this study are available from the corresponding author upon reasonable request. However, due to data sensitivity, a full dataset will not be publicly available.

Code availability

Not applicable.

Abbreviations

ACT:

Activated clotting time

AF:

Atrial fibrillation

CT:

Computed tomography

DAPT:

Dual antiplatelet therapy

DOAC:

Direct oral anticoagulation

ICE:

Intracardiac echocardiography

IU:

International units

LAAO:

Left atrial appendage occlusion

LMWH:

Low molecular weight heparin

TEE:

Transoesophageal echocardiography

TTE:

Transthoracic echocardiography

TIA:

Transient ischemic attack

References

  1. Hindricks G et al. 2020 ESC Guidelines for the diagnosis and management of atrial fibrillation developed in collaboration with the European Association for Cardio-Thoracic Surgery (EACTS): The Task Force for the diagnosis and management of atrial fibrillation of the European Society of Cardiology (ESC) Developed with the special contribution of the European Heart Rhythm Association (EHRA) of the ESC. Eur Heart J. 2021;42(5):373–498.

  2. Lane DA, et al. Temporal trends in incidence, prevalence, and mortality of atrial fibrillation in primary care. J Am Heart Assoc. 2017;6(5).

  3. Gadsbøll K, et al. Increased use of oral anticoagulants in patients with atrial fibrillation: temporal trends from 2005 to 2015 in Denmark. Eur Heart J. 2017;38(12):899–906.

    PubMed  Google Scholar 

  4. Osmancik P, et al. Left atrial appendage closure versus direct oral anticoagulants in high-risk patients with atrial fibrillation. J Am Coll Cardiol. 2020;75(25):3122–35.

    Article  CAS  Google Scholar 

  5. Reddy VY, et al. 5-Year outcomes after left atrial appendage closure: from the PREVAIL and PROTECT AF Trials. J Am Coll Cardiol. 2017;70(24):2964–75.

    Article  Google Scholar 

  6. Holmes DR, et al. Percutaneous closure of the left atrial appendage versus warfarin therapy for prevention of stroke in patients with atrial fibrillation: a randomised non-inferiority trial. Lancet. 2009;374(9689):534–42.

    Article  CAS  Google Scholar 

  7. Nielsen-Kudsk JE, et al. Clinical outcomes associated with left atrial appendage occlusion versus direct oral anticoagulation in atrial fibrillation. JACC Cardiovasc Interv. 2021;14(1):69–78.

    Article  Google Scholar 

  8. Ferraris VA, et al. Consensus review of the treatment of cardiovascular disease in people with hemophilia A and B. Cardiol Rev. 2015;23(2):53–68.

    Article  Google Scholar 

  9. Schutgens RE, et al. Atrial fibrillation in patients with haemophilia: a cross-sectional evaluation in Europe. Haemophilia. 2014;20(5):682–6.

    Article  CAS  Google Scholar 

  10. Schutgens RE, et al. Management of atrial fibrillation in people with haemophilia–a consensus view by the ADVANCE Working Group. Haemophilia. 2014;20(6):e417–20.

    Article  CAS  Google Scholar 

  11. Martin K, Key NS. How I treat patients with inherited bleeding disorders who need anticoagulant therapy. Blood. 2016;128(2):178–84.

    Article  CAS  Google Scholar 

  12. Korsholm K, Jensen JM, Nielsen-Kudsk JE. Cardiac computed tomography for left atrial appendage occlusion: acquisition, analysis, advantages, and limitations. Interv Cardiol Clin. 2018;7(2):229–42.

    PubMed  Google Scholar 

  13. Korsholm K, et al. Expert recommendations on cardiac computed tomography for planning transcatheter left atrial appendage occlusion. JACC Cardiovasc Interv. 2020;13(3):277–92.

    Article  Google Scholar 

  14. Korsholm K, Jensen JM, Nielsen-Kudsk JE. Intracardiac echocardiography from the left atrium for procedural guidance of transcatheter left atrial appendage occlusion. JACC Cardiovasc Interv. 2017;10(21):2198–206.

    Article  Google Scholar 

  15. Tzikas A, et al. Left atrial appendage occlusion for stroke prevention in atrial fibrillation: multicentre experience with the AMPLATZER Cardiac Plug. EuroIntervention. 2016;11(10):1170–9.

    Article  Google Scholar 

  16. Nielsen-Kudsk JE, et al. Left atrial appendage occlusion versus standard medical care in patients with atrial fibrillation and intracerebral haemorrhage: a propensity score-matched follow-up study. EuroIntervention. 2017;13(3):371–8.

    Article  Google Scholar 

  17. Sümnig A, et al. Acquired hemophilia with inhibitors presenting as an emergency: misinterpretation of clotting results during direct oral anticoagulation. Dtsch Arztebl Int. 2014;111(19):345–8.

    PubMed  PubMed Central  Google Scholar 

  18. Lövdahl S, et al. Hypertension and cardiovascular diseases in Swedish persons with haemophilia - a longitudinal registry study. Thromb Res. 2019;181:106–11.

    Article  Google Scholar 

  19. Sharathkumar AA, et al. Prevalence and risk factors of cardiovascular disease (CVD) events among patients with haemophilia: experience of a single haemophilia treatment centre in the United States (US). Haemophilia. 2011;17(4):597–604.

    Article  CAS  Google Scholar 

  20. Boersma LV, et al. Implant success and safety of left atrial appendage closure with the WATCHMAN device: peri-procedural outcomes from the EWOLUTION registry. Eur Heart J. 2016;37(31):2465–74.

    Article  Google Scholar 

  21. Hildick-Smith D, et al. Left atrial appendage occlusion with the AmplatzerTM AmuletTM device: full results of the prospective global observational study. Eur Heart J. 2020;41(30):2894–901.

    Article  CAS  Google Scholar 

  22. Freitas-Ferraz AB, et al. Safety of transesophageal echocardiography to guide structural cardiac interventions. J Am Coll Cardiol. 2020;75(25):3164–73.

    Article  Google Scholar 

  23. Natale A, et al. Venous vascular closure system versus manual compression following multiple access electrophysiology procedures: the AMBULATE Trial. JACC Clin Electrophysiol. 2020;6(1):111–24.

    Article  Google Scholar 

  24. Lempereur M, et al. Device-associated thrombus formation after left atrial appendage occlusion: a systematic review of events reported with the Watchman, the Amplatzer Cardiac Plug and the Amulet. Catheter Cardiovasc Interv. 2017;90(5):E111-e121.

    Article  Google Scholar 

  25. Main ML, et al. Assessment of device-related thrombus and associated clinical outcomes with the WATCHMAN left atrial appendage closure device for embolic protection in patients with atrial fibrillation (from the PROTECT-AF Trial). Am J Cardiol. 2016;117(7):1127–34.

    Article  Google Scholar 

  26. Enomoto Y, et al. Use of non-warfarin oral anticoagulants instead of warfarin during left atrial appendage closure with the Watchman device. Heart Rhythm. 2017;14(1):19–24.

    Article  Google Scholar 

  27. Dannenberg L, et al. Antiplatelet effects of aspirin and clopidogrel after left atrial appendage (LAA) occluder implantation. Int J Cardiol. 2019;275:95–100.

    Article  Google Scholar 

  28. Korsholm K, et al. Transcatheter left atrial appendage occlusion in patients with atrial fibrillation and a high bleeding risk using aspirin alone for post-implant antithrombotic therapy. EuroIntervention. 2017;12(17):2075–82.

    Article  Google Scholar 

  29. Güray Ü, et al. Percutaneous left atrial appendage closure in a patient with haemophilia and atrial fibrillation: a case report. Eur Heart J Case Rep. 2019;3(3):ytz124.

  30. Cheung VT, et al. Management of thromboembolic risk in persons with haemophilia and atrial fibrillation: is left atrial appendage occlusion the answer for those at high risk? Haemophilia. 2013;19(2):e84–6.

    Article  CAS  Google Scholar 

  31. Toselli M, et al. Left atrial appendage closure: a balanced management of the thromboembolic risk in patients with hemophilia and atrial fibrillation. J Thromb Thrombolysis. 2020;50(3):668–73.

    Article  CAS  Google Scholar 

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Funding

ADK is currently enrolled as a PhD fellow at Aarhus University and has received a full scholarship from the institution. The project did not receive further funding.

Author information

Authors and Affiliations

Authors

Contributions

All the authors contributed to the study conception and design. Data collection and analysis was performed by ADK, KK, and AK. The first draft of the manuscript was written by ADK and all the authors critically revised several versions of the manuscript. Lastly, all the authors read and approved the final manuscript.

Corresponding author

Correspondence to Anders Dahl Kramer.

Ethics declarations

Ethics approval

The study was approved by the Danish Patient Safety Authority (3–3013-2736/1) and Central Denmark Region (1–45-70–60-20) and carried out in accordance with the Declaration of Helsinki.

Consent to participate

All involved patients provided informed consent prior to intervention.

Conflict of interest

ADK has nothing to disclose. KK has received lecture fees from Abbott. AK has received advisory board fees and lecture fees from Bayer and AstraZeneca. LHP has been an investigator on clinical trials from Bayer Health Care and Novo Nordisk. JENK is a proctor for Abbott and Boston Scientific.

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Kramer, A.D., Korsholm, K., Kristensen, A. et al. Left atrial appendage occlusion in haemophilia patients with atrial fibrillation. J Interv Card Electrophysiol 64, 95–102 (2022). https://doi.org/10.1007/s10840-021-01090-z

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  • DOI: https://doi.org/10.1007/s10840-021-01090-z

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