Management of Patients with Congenital Bleeding Disorders and Cardiac Indications for Antithrombotic Therapy

Authors: Atar, D; Vandenbriele, C; Agewall, S; Gigante, B; Goette, A; Gorog, DA; Holme, PA; Krychtiuk, KA; Rocca, B; Siller-Matula, JM; Valgimigli, M; Rubboli, A; Klamroth, R.

Affiliations: Div. of Cardiology, Oslo University Hospital Ulleval, Oslo, Norway. Institute of Clinical Medicine, University of Oslo, Norway. Heart Center, OLV Hospital, Aalst, Belgium. Royal Brompton & Harefield hospitals, Guy’s & St. Thomas’ NHS Foundation Trust, London, UK. Division of Clinical Science, Danderyd hospital, Karolinska Institute Stockholm, Sweden. Unit of Cardiovascular Medicine, Department of Medicine, Karolinska Institutet, Stockholm, Sweden. Department of Cardiology, Danderyd hospital, Stockholm, Sweden. Dept. of Cardiology and Intensive Care Medicine, St. Vincenz Hospital, Paderborn, Germany. Otto-von-Guericke University, Medical Faculty, Magdeburg, Germany. MAESTRIA Consortium, AFNET, Münster, Germany. Faculty of Medicine, National Heart and Lung Institute, Imperial College, London, United Kingdom School of Life. Medical Sciences, Postgraduate Medical School, University of Hertfordshire, Hatfield, Hertfordshire, UK. Institute of Clinical Medicine, University of Oslo, Norway. Department of Haematology, Oslo University Hospital, Oslo, Norway. Department of Internal Medicine II-Division of Cardiology, Medical University of Vienna, Vienna, Austria. Department of Medicine and Surgery, LUM University, Casamassima, Bari, Italy. Department of Internal Medicine II-Division of Cardiology, Medical University of Vienna, Vienna, Austria. Cardiocentro Ticino Institute, Ente Ospedaliero Cantonale, Lugano, Switzerland. Department of Cardiology, Bern University Hospital, University of Bern, Bern, Switzerland. Department of Emergency, Internal Medicine and Cardiology, Division of Cardiology, S. Maria delle Croci Hospital, Ravenna, Italy. Institute of Clinical Medicine, University of Oslo, Norway. Department for Internal Medicine, Haemophilia Treatment Centre, Vivantes Klinikum im Friedrichshain, Berlin, Germany. Institute of Experimental Hematology and Transfusion Medicine, University Hospital Bonn, Medical Faculty, University of Bonn, Bonn, Nordrhein-Westfalen, Germany.

Publication: European heart journal. Cardiovascular pharmacotherapy. 2025.

ABSTRACT: Cardiologists have only had rare exposure to haemophilia patients and patients with other congenital bleeding disorders during the last decades, as these patients had a reduced life expectancy and were partly protected against thrombosis due to the bleeding disorder. With the availability of effective and safe replacement therapies of clotting factors, the average life expectancy in these populations of patients has significantly increased, and thrombotic complications may occur. The European Society of Cardiology (ESC) Working Group on Thrombosis has taken the initiative to broaden the spectrum of these haematological conditions to include patients with a larger variety of congenital bleeding disorders with concomitant cardiac conditions as compared to a recent position paper by the European Haematology Association (EHA) in collaboration with other societies (ISTH, EAHAD, ESO). The management of antithrombotic therapy or thromboprophylaxis in these individuals is challenging due to the wide phenotypes encompassed by congenital bleeding disorders. These include abnormalities in both primary haemostasis (involving von Willebrand factor and platelet function) and secondary haemostasis (related to coagulation factors and fibrinogen). Bleeding disorders range from mild to very severe. Based on existing literature, we provide clinical consensus statements on optimizing antithrombotic treatment strategies for patients with congenital bleeding disorders and highlight the current gaps in knowledge in these complex clinical settings. Of importance, an individualized approach to antithrombotic therapy is warranted to properly balance the two risks of thrombosis and bleeding. Adoption of the safest interventional techniques, reduction of the intensity and/or duration of antithrombotic therapies, and attention to the safe levels of clotting factors is generally advised.