TY - JOUR
T1 - Reduced cardiovascular morbidity in patients with hemophilia
T2 - results of a 5-year multinational prospective study
AU - Van Der Valk, Paul
AU - Makris, Michael
AU - Fischer, Kathelijn
AU - Tait, Robert C
AU - Chowdary, Pratima
AU - Collins, Peter W
AU - Meijer, Karina
AU - van Vulpen, Lize F D
AU - Mauser-Bunschoten, Eveline
AU - Schutgens, Roger E G
N1 - Funding Information:
research grants from Bayer, Novo Nordisk, Octapharma, and Sobi (all outside this work, all payments made to institution). The remaining author declares no competing financial interests.
Funding Information:
The central coordination of this study was supported by an investigator-initiated research grant (no. H15-29843) from Baxalta US Inc., a member of the Takeda group of companies. Takeda had no influence in the design, execution of the study, or the manuscript.
Funding Information:
The central coordination of this study was supported by an investigator-initiated research grant (no. H15-29843) from Bax-alta US Inc., a member of the Takeda group of companies.
Publisher Copyright:
© 2022 by The American Society of Hematology.
PY - 2022/2/8
Y1 - 2022/2/8
N2 - Hemophilia is a congenital bleeding disorder caused by low levels of clotting factor VIII or IX. The life expectancy of people with hemophilia (PWH) has increased with the availability of clotting factor concentrates. At the same time, the incidence of cardiovascular disease (CVD) has increased; in retrospective studies, there are conflicting data regarding if, despite this increase, the incidence is still lower than in the general population. We prospectively compared the incidence of CVD in PWH vs the predicted incidence. This prospective, multicenter, observational study included adult PWH (aged >30 years) from The Netherlands and United Kingdom. They were followed up for a 5-year period, and CVD incidence was compared with a predicted event rate based on the QRISK2-2011 CVD risk model. The primary end point was the observed fatal and nonfatal CVD incidence after 5 years compared with the estimated events and in relation to severity of hemophilia. The study included 709 patients, of whom 687 (96.9%) completed 5 years' follow-up or reached an end point. For 108 patients, the QRISK score could not be calculated at inclusion. For the remaining 579, fewer CVD events were observed than predicted: 9 vs 24 (relative risk, 0.38; 95% confidence interval, 0.18-0.80; P = .01), corresponding with an absolute risk reduction of 2.4%. Severe hemophilia treated on demand had the highest risk reduction. There was no statistically significant relation between severity of hemophilia and incidence of CVD. In hemophilia, a lower-than-predicted CVD incidence was found, supporting the theory that hemophilia protects against CVD. The study is registered at www.clinicaltrials.gov as #NCT01303900.
AB - Hemophilia is a congenital bleeding disorder caused by low levels of clotting factor VIII or IX. The life expectancy of people with hemophilia (PWH) has increased with the availability of clotting factor concentrates. At the same time, the incidence of cardiovascular disease (CVD) has increased; in retrospective studies, there are conflicting data regarding if, despite this increase, the incidence is still lower than in the general population. We prospectively compared the incidence of CVD in PWH vs the predicted incidence. This prospective, multicenter, observational study included adult PWH (aged >30 years) from The Netherlands and United Kingdom. They were followed up for a 5-year period, and CVD incidence was compared with a predicted event rate based on the QRISK2-2011 CVD risk model. The primary end point was the observed fatal and nonfatal CVD incidence after 5 years compared with the estimated events and in relation to severity of hemophilia. The study included 709 patients, of whom 687 (96.9%) completed 5 years' follow-up or reached an end point. For 108 patients, the QRISK score could not be calculated at inclusion. For the remaining 579, fewer CVD events were observed than predicted: 9 vs 24 (relative risk, 0.38; 95% confidence interval, 0.18-0.80; P = .01), corresponding with an absolute risk reduction of 2.4%. Severe hemophilia treated on demand had the highest risk reduction. There was no statistically significant relation between severity of hemophilia and incidence of CVD. In hemophilia, a lower-than-predicted CVD incidence was found, supporting the theory that hemophilia protects against CVD. The study is registered at www.clinicaltrials.gov as #NCT01303900.
UR - http://www.scopus.com/inward/record.url?scp=85124329639&partnerID=8YFLogxK
U2 - 10.1182/bloodadvances.2021005260
DO - 10.1182/bloodadvances.2021005260
M3 - Article
C2 - 34879394
SN - 2473-9529
VL - 6
SP - 902
EP - 908
JO - Blood Advances
JF - Blood Advances
IS - 3
ER -