Left atrial appendage closure: a balanced management of the thromboembolic risk in patients with hemophilia and atrial f

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Left atrial appendage closure: a balanced management of the thromboembolic risk in patients with hemophilia and atrial fibrillation Marco Toselli1 · Davide Bosi1 · Giorgio Benatti1 · Emilia Solinas1 · Maria Alberta Cattabiani1 · Luigi Vignali1

© Springer Science+Business Media, LLC, part of Springer Nature 2020

Abstract Atrial fibrillation is the most common cardiac arrhythmia and is a major cause of embolic stroke. In patients at high bleeding risk such as those with hemophilia, the thromboembolic prevention is challenging. We herein present three patients affected by moderate to severe hemophilia and atrial fibrillation that were treated in our Institution between March 2018 and September 2019, with percutaneous left atrial appendage closure. In patients with relative or absolute contraindications to long-term anticoagulant therapy, the left atrial appendage closure could represent a reasonable strategy. Keywords  Atrial fibrillation · Hemophilia · Left atrial appendage closure · Case series

Highlights • Patients with hemophilia and atrial fibrillation are a chal• •





lenging population being at high bleeding and thrombosis risk Left atrial appendage closure guarantees stroke prevention avoiding oral anticoagulant therapy in patients at high bleeding risk A multidisciplinary team (hematologists, clinical and interventional cardiologists) discussion has a central role in the diagnostic-therapeutic management. A strict follow up is recommended After the procedure the period of dual antiplatelet therapy should be kept as short as possible and prophylactic factor replacement may be indicated depending on hemophilia severity Transcatheter procedure seems to be cost-effective in comparison with lifelong oral anticoagulant therapy and clotting factor replacement.

* Marco Toselli [email protected] 1



Azienda Ospedaliera-Universitaria di Parma – Interventional Cardiology Unit, Viale Gramsci 14, 43126 Parma, Italy

Introduction Atrial fibrillation (AF) is the most frequent arrhythmia in the elderly population and it represents the major risk factor for cardio-embolic events, especially cerebral stroke [1]. One of the first issues that a physician has to face when a new diagnosis of AF is done is to evaluate the antithrombotic strategy balancing the ischemic and bleeding risks with appropriate scores (i.e. CHA2DS2-VASc and HAS-BLED). However, in patients at high bleeding risk (HBR) the decision making is challenging, being a heterogenous population with some relative or absolute contraindications to anticoagulant medications. Patients at HBR include those that are affected by hemophilia. Hemophilia is a hereditary bleeding disorder caused by the deficiency of a coagulation factor: hemophilia A is characterized by factor VIII deficiency while hemophilia B is due to the deficit of factor IX. Hemophilia is a X-linked recessive disorder that typically affects male gender. The deficiency of the coagulation factors leads to a bleeding predisposition. Indeed, patients with haemophilia (PWH) may experience spo