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Cardiovascular disease‐related hospitalization and mortality among persons with von Willebrand disease: A nationwide register study in Sweden
Author(s) -
Holm Elena,
Osooli Mehdi,
Steen Carlsson Katarina,
Berntorp Erik
Publication year - 2019
Publication title -
haemophilia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.213
H-Index - 92
eISSN - 1365-2516
pISSN - 1351-8216
DOI - 10.1111/hae.13642
Subject(s) - medicine , disease , population , hazard ratio , pediatrics , diabetes mellitus , von willebrand disease , demography , von willebrand factor , confidence interval , environmental health , platelet , sociology , endocrinology
It has been hypothesized that persons with von Willebrand disease (VWD) may be protected against arterial thrombosis despite having atherosclerosis. Aim To calculate a nationwide estimate of the absolute and comparative burden of cardiovascular disease (CVD) hospitalization and mortality among persons with VWD using birthdate and sex‐matched comparisons from the general population in Sweden. Methods Persons with VWD regardless of the type and severity, diagnosed by a medical doctor, who lived in Sweden for some time during the observation period 1987 through 2008 were included. For each participant with VWD, up to five randomly selected birthdate‐ and sex‐matched persons from general population were selected as controls. Results A total of 2790 participants with VWD including 888 male and 1902 female subjects and 13 938 controls were included. Overall, the hazard of CVD‐related hospitalization was 1.3‐fold (95% CI: 1.1, 1.5) among participants with VWD after adjusting for sex, birthdate, diabetes and cancer. However, they had a 0.4‐fold (95% CI: 0.3, 0.6) hazard of CVD‐related mortality compared to general population sample. Conclusions In this nationwide, long‐term register study with individually matched controls, we have been able to show that persons with VWD have a higher hospitalization rate due to CVD events. However, the mortality rates appear lower than in the control population. The latter finding is consistent with previous studies and indicates a protective effect of the clotting factor deficiency inherited with VWD.