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Treatment of thrombotic cardiovascular diseases in people with haemophilia: A Japanese consensus study
Azusa Nagao1, Yushi Chikasawa2, Yukio Hiroi3
1Department of Blood Coagulation, Ogikubo Hospital, Tokyo, Japan.
Insights
This study developed expert guidance for managing cardiovascular diseases (CVD) in people with haemophilia (PWH) in Japan, addressing a critical gap in current clinical practice for this complex patient group.
Area of Science:
- Cardiology
- Haematology
- Clinical Guideline Development
Background:
- Cardiovascular diseases (CVD) pose management challenges for people with haemophilia (PWH) requiring anticoagulant/antiplatelet therapy alongside factor replacement.
- A significant unmet need exists for Japanese guidelines on managing PWH with CVD.
Purpose of the Study:
- To establish expert consensus-based guidance for the management of CVD in people with haemophilia (PWH) in Japan.
- To provide a framework for cardiologists and haematologists treating PWH with cardiovascular conditions.
Main Methods:
- A steering committee identified key themes and developed 44 statements for an online questionnaire.
- Expert specialists in PWH and CVD management in Japan responded to Likert scale and multiple-choice questions.
- Consensus was defined by high (≥75%) or very high (≥90%) agreement among respondents.
Main Results:
- Fifteen out of sixteen invited specialists participated in the consensus study.
- A high level of agreement (≥90%) was achieved for 71% of statements, with strong agreement (75%-89%) for an additional 17%.
- Specific target clotting factor levels were agreed upon for managing CVD in PWH, including in cases of atrial fibrillation or myocardial infarction.
Conclusions:
- The consensus study provides a foundational framework for managing PWH with CVD in Japan.
- Implementing these recommendations is expected to enhance clinical outcomes for PWH experiencing cardiovascular diseases.
Introduction:
Cardiovascular diseases (CVD) that require long-term anticoagulant and antiplatelet therapy presents a problem in people with haemophilia (PWH) who receive factor replacement therapy to reduce bleeding risk. Currently, there are no Japanese guidelines for the management of 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 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. Agreement on specific target trough clotting factor levels for managing certain clinical situations, such as when in the presence of non-valvular atrial fibrillation or myocardial infarction, was also achieved.
Conclusion:
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.
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