Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2024 Jul;30(4):933-942.
doi: 10.1111/hae.15039. Epub 2024 May 23.

Treatment of thrombotic cardiovascular diseases in people with haemophilia: A Japanese consensus study

Azusa Nagao  1 , Yushi Chikasawa  2 , Yukio Hiroi  3 , Masahiro Ieko  4
Affiliations

Treatment of thrombotic cardiovascular diseases in people with haemophilia: A Japanese consensus study

Azusa Nagao et al. Haemophilia. 2024 Jul.

Abstract

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.

Keywords: Japan; cardiovascular diseases; consensus; haemophilia; practice guideline.

PubMed Disclaimer

References

REFERENCES

    1. von Drygalski A, Chowdary P, Kulkarni R, et al. XTEND‐1 Trial group. Efanesoctocog alfa prophylaxis for patients with severe hemophilia A. N Engl J Med. 2023;388:310‐318.
    1. S&P Global. Roche's hemophilia therapy Hemlibra gets Japanese approval [Internet]. 2018. Accessed November 2023. Available from: https://www.spglobal.com/marketintelligence/en/news‐insights/trending/fl...
    1. Matsushita T, Shapiro A, Abraham A, et al. Phase 3 trial of concizumab in hemophilia with inhibitors. N Engl J Med. 2023;389(9):783‐794. explorer7 Investigators.
    1. Ministry of Health, Labour and Welfare. Nationwide Survey on Coagulation Disorders 2022 [Internet]. 2023. Published by Japan Foundation for AIDS Prevention. Accessed October 2023. Available from: https://api‐net.jfap.or.jp/library/project.html
    1. Nakatani H. Population aging in Japan: policy transformation, sustainable development goals, universal health coverage, and social determinates of health. Glob Health Med. 2019;1(1):3‐10.

LinkOut - more resources