Purpose: Aortic aneurysms have a high fatality rate that could be reduced with control of risk factors and use of available screening methods for detection of early changes in aortic walls. The available data on familial risks, a potential indication for screening, are mainly limited to abdominal aortic aneurysms.
Methods: A nationwide Swedish cohort was constructed by linking the Multigeneration Register on 0- to 69-year-old siblings to the Hospital Discharge Register and the Cause of Death Register for data on aortic aneurysms from years 1987 to 2001. Standardized incidence ratios (SIRs) were calculated for affected siblings by comparing with those whose siblings had no aneurysm.
Results: A total of 71 affected siblings were identified with a familial SIR of 8.71; when one sibling was diagnosed before age 50 years, the SIR was 19.69. For concordant thoracic or concordant abdominal aneurysms, the SIRs were 21.68 and 13.06, respectively. For brothers, the risk of abdominal aneurysms was 14.63, and 49.50 for diagnosis before age 50 years. Familial risks and the effects of early diagnostic age were shared by the anatomic subtypes of aneurysms. Within limits of the sample size, no gender differences could be observed. Affected siblings constituted 2.2% of all diagnosed patients.
Conclusions: A family history of any aortic aneurysms and age groups younger than 50 years should be considered in recommendations for screening. The high familial risks are likely to be the result of heritable genes, the identification of which would allow gene testing and preventive counseling.