PB0495 - Treatment of Thrombotic Cardiovascular Diseases in People with Haemophilia: A Japanese Consensus Study

Background: Cardiovascular diseases (CVD) that require long-term anticoagulant or antiplatelet therapy present particular challenges for Japanese people with haemophilia (PWH), who are known to have an increased tendency to bleed. This characteristic significantly complicates the direct application of Western guidelines to Japanese PWH. Nonetheless, there are currently no specific guidelines in Japan for managing PWH with CVD.

Aims: To develop expert guidance on managing CVD in PWH in Japan.

Methods: A steering committee of four experts (two haemophilia specialists, one thrombosis specialist, one cardiologist) identified 44 statements related to five key themes. An online questionnaire was produced comprising a mix of 4-point Likert scale and multiple-choice questions that was sent to specialists in the management of PWH with CVD in Japan. Consensus was defined as high or very high if a respective ≥75% or ≥90% of respondents agreed with a statement.

Results: Of 16 potential respondents, responses were received from 15 specialists. Of the Likert scale questions, 71% (29/41) achieved ≥90% agreement (very strong agreement), 17% (7/41) achieved 75%–89% agreement (strong agreement), and 15% (6/41) did not achieve consensus agreement. The three multiple-choice questions failed to identify a strong preference. Ultimately, recommendations were made for five major categories (figures). Among them, 'Managing Atrial Fibrillation' and 'Managing Myocardial Infarction' are shown in the figure 1. The others are 'Identifying Cardiovascular Disease Risk', 'Managing Angina', and 'Principles of Decision Making in Managing Cardiovascular Disease Risk'."

Conclusion(s): The results of this consensus study provide a framework for cardiologists and haematologists to manage PWH who are at risk of, or who have, CVD. Implementation of the recommendations provided herein may improve outcomes for PWH with CVD, as they provide guidance on specific target trough clotting factor levels for different clinical situations, the role of non-factor replacement therapies, and the organisation of care.