Abstract Number: PB0583
Meeting: ISTH 2021 Congress
Theme: Hemophilia and Rare Bleeding Disorders » Hemophilia - Clinical
Background: The management of atrial fibrillation (AF) in persons with hemophilia is a challenge as antithrombotic therapy, the standard of care, is usually contraindicated. Left atrial appendage occlusion (LAAO) is an alternative option for patients with a contraindication to antithrombotic therapy.
Aims: We present the case of a person with mild hemophilia A (HA) and AF who underwent successful LAAO with short-term warfarin therapy, and a systematic review of existing cases in the literature.
Methods: Case report and a systematic search in Embase, Scopus and PubMed for existing cases in the literature.
Results: A 79-year-old man with mild HA (baseline factor VIII (FVIII) levels 9%) presented with a transient ischemic attack (TIA) secondary to rate-controlled AF. He was previously on low-dose aspirin with no bleeding complications. He did not require treatment for his TIA as his neurologic symptoms resolved within 24 hours. His CHADS2-VASc risk score was 7 and HAS-BLED score was 5. LAAO was recommended along with short-term warfarin therapy for 6 weeks post-procedure. He received FVIII replacement therapy with a target of 100% pre-procedure. Post-procedure, warfarin (goal INR 2-3) was initiated without a heparin bridge. Aspirin was discontinued. Post-procedure, he received bolus FVIII infusions to maintain trough FVIII levels of 30% while on warfarin therapy. At 6 weeks, warfarin and factor replacement therapy were discontinued, and low-dose aspirin was resumed. He remains well at 12-months follow-up with no bleeding or thrombotic complications.
A systematic review of the literature identified an additional 8 cases of persons with hemophilia who underwent LAAO. The clinical characteristics and outcomes of these cases are shown in Table 1.
Author (year) | CHA₂DS₂-VASc | Age/ Gender |
Prophylaxis therapy | Factor deficiency | Diagnosis | Replacement therapy during and post-implantation | Anti-thrombotic therapy post-intervention | Short-term outcome | Duration of follow-up |
---|---|---|---|---|---|---|---|---|---|
Cheung (2013) | 6 | 73M | None | Factor VIII (8%) | CABG, stroke, carotid endarterectomy, AF. Had previously tolerated SAPT (Aspirin or Clopidogrel) without any bleeding complications | ≥100% pre-procedure, then ≥80% trough for 3 days, then >30% while on DAPT | Aspirin 75 mg and clopidogrel 75 mg for 6 weeks | TOE at 6 weeks showed well-sealed device. Aspirin and factor infusion discontinued, continued clopidogrel life-long | 9 months |
Bhatti (2019) | 3 | 60F | None | Factor IX (15%) | Paroxysmal AF, Sick sinus syndrome, s/p pacemaker implantation, TIA | 82 U/kg of factor pre-procedure, then 67 U/kg on POD #1, then 20 U/kg every other day to maintain levels >30% | Warfarin (INR 2-3) |
TOE at 30 days showed well-sealed device with no leak. Warfarin and factor infusion discontinued | 6 months |
Guray (2019) | 3 | 67M | On prophylaxis to keep FVIII at 10% | Factor VIII (NR) | AF | Factor pre-procedure and post-procedure | Aspirin 81 mg and clopidogrel 75 mg for 1 month with factor infusion, then only aspirin 81 mg for 2 more months | TOE at 6 weeks showed well-sealed device. All antithrombotic therapy discontinued at 3 months | 1 year |
Coppola (2020) | 3 | M | Tertiary prophylaxis | Factor VIII (<1%) | AF | ≥80% pre-procedure and for 12 hours post-procedure, then continued factor infusion to maintain trough >5% | Clopidogrel | Withdrew clopidogrel after 2 months due to severe epistaxis and joint bleeds | NR |
3 | M | Tertiary prophylaxis | Factor VIII (<1%) | AF | ≥80% pre-procedure and for 12 hours post-procedure, then continued factor infusion to maintain trough >5% | Clopidogrel | NR | NR | |
Toselli (2020) | 3 | 76M | Tertiary prophylaxis | Factor VIII (<1%) | Asymptomatic AF | >60% pre-procedure, then resumed usual prophylaxis | 300 mg clopidogrel loading dose, then clopidogrel 75 mg | Within the first few weeks, FVIII dosing was increased due to minor hemarthrosis and epistaxis. TOE at 3 months showed well-sealed device. Clopidogrel discontinued. Usual factor prophylaxis dosing resumed | 20 months |
4 | 73M | None | Factor IX (Moderate) | CABG, ischemic cardiomyopathy, residual heart failure with LVEF 40%, paroxysmal AF | 40 U/kg of factor infused pre-procedure | DAPT for 3 months, then SAPT life-long | TOE at 6 months showed well-sealed device | 12 months | |
5 | 79M | Tertiary prophylaxis | Factor VIII (<1%) | TIA, recurrent AF | >65% pre-procedure, then resumed usual prophylaxis | Clopidogrel 75 mg | Cardiac CT at 3 months showed optimal device position, clopidogrel discontinued | NR | |
This case (2021) | 7 | 79M | None | Factor VIII (9%) | CAD, s/p PCI, AF s/p AVN ablation and CRT-P placement, stroke (on aspirin 81 mg) | >100% pre-procedure, then continued factor infusion to maintain trough >30% | Warfarin (INR 2-3) |
TOE at 6 weeks showed well-sealed device. Warfarin discontinued, remained on aspirin 81 mg | 12 months |
Conclusions: Our case report and systematic review demonstrate that with appropriate hemostatic management, LAAO can be a safe and successful strategy for reducing the long-term thromboembolic risk of persons with hemophilia and AF.
To cite this abstract in AMA style:
Lim MY, Abou-Ismail MY. Left Atrial Appendage Occlusion for the Management of Atrial Fibrillation in Persons with Hemophilia: A Case Report and Systematic Review of the Literature [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/left-atrial-appendage-occlusion-for-the-management-of-atrial-fibrillation-in-persons-with-hemophilia-a-case-report-and-systematic-review-of-the-literature/. Accessed April 19, 2024.« Back to ISTH 2021 Congress
ISTH Congress Abstracts - https://abstracts.isth.org/abstract/left-atrial-appendage-occlusion-for-the-management-of-atrial-fibrillation-in-persons-with-hemophilia-a-case-report-and-systematic-review-of-the-literature/