Cardiovascular disease-related hospitalization and mortality among persons with von Willebrand disease: A nationwide register study in Sweden.

INTRODUCTION: 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.