Introduction: Cardiovascular diseases (CVD) requiring long-term anticoagulant and antiplatelet therapy pose challenges in people with haemophilia (PWH) on factor replacement therapy. Japanese PWH have a predisposition to a higher risk of bleeding, but there are currently no guidelines for managing Japanese PWH with CVD.

Aim: 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 on the management of CVD in PWH and sent an online questionnaire to 16 Japanese specialists. Consensus with ≥75% and ≥90% of the respondents was defined as high and very high agreement, respectively.

Results: Responses were received from 15 of 16 specialists. Of the 41 Likert scale questions, 29 achieved very high agreement, 7 achieved high agreement, and 6 did not reach a consensus. The three multiple-choice questions did not identify strong preferences. Specific target trough clotting factor levels for managing specific clinical situations, such as non-valvular atrial fibrillation or myocardial infarction, achieved consensus.

Conclusion: This consensus study provides a framework for cardiologists and haematologists to manage PWH with CVD. Implementing these recommendations may improve the outcomes for PWH with CVD.