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Family History and Aortic Dissection Incidence, Outcomes
Quick Takes
- A family history of aortic dissection (AD) was associated with a >6-fold increased risk of AD.
- Patients with a family history of AD presented at a younger age compared to those without a family history.
- Patients with AD and a family history of AD had a higher risk of undergoing aortic surgery compared to those without a family history.
Study Questions:
Are the incidence and prognosis of aortic dissection (AD) influenced by family history of AD?
Methods:
The Taiwan National Health Insurance database was used to perform both cross-sectional and cohort studies. A registry parent–offspring relationship algorithm was used to reconstruct the genealogy of this population for heritability estimation. The cross-sectional study included 23,868 patients with a diagnosis of AD in 2015. The prevalence and adjusted relative risks (RRs) were evaluated, and the liability threshold model was used to examine the effects of heritability and environmental factors. A 1:10 propensity score-matched cohort comprising AD patients with or without a family history of AD was included to compare late outcomes in the cohort study.
Results:
A family history of AD in first-degree relatives was associated with a RR of 6.82 (95% confidence interval [CI], 5.12-9.07). The heritability of AD was estimated to be 57.0% for genetic factors, and 3.1% and 40.0% for shared and nonshared environmental factors, respectively. After excluding individuals with Marfan syndrome or bicuspid aortic valve, a family history of AD was associated with a RR of 6.56 (95% confidence interval [CI], 4.92-8.77) for AD. Patients with AD and a family history of AD presented at a younger age compared to those without a family history of AD (51.5 years vs. 59.7 years), and patients with AD and a family history of AD had a higher risk of undergoing aortic surgery compared to those without a family history (subdistribution hazard ratio, 1.40; 95% CI, 1.12-1.76).
Conclusions:
A family history of AD was a strong risk factor for AD. Patients with AD and a family history of AD had a higher risk of later aortic surgery than did those with no family history of AD.
Perspective:
AD is a relatively uncommon but life-threatening condition. Known risk factors include hypertension; smoking; vascular inflammation; and genetic disorders including Marfan syndrome, Ehlers-Danlos, Turner’s, Loeys-Dietz, and bicuspid aortic valve. This large administrative database study found a >6-fold increased risk of AD among people with a family history of AD, with contributions from genetic and both shared and unshared environmental factors. This study supports that, among patients with a family history of AD, aggressive risk factor modification should be pursued, including smoking avoidance or cessation, and hypertension control; and it may be reasonable to consider early consultation for genetic disorders, and routine screening imaging examinations.
Clinical Topics: Arrhythmias and Clinical EP, Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Invasive Cardiovascular Angiography and Intervention, Prevention, Valvular Heart Disease, Vascular Medicine, Genetic Arrhythmic Conditions, Aortic Surgery, Cardiac Surgery and Arrhythmias, Cardiac Surgery and CHD and Pediatrics, Cardiac Surgery and VHD, Congenital Heart Disease, CHD and Pediatrics and Arrhythmias, CHD and Pediatrics and Interventions, CHD and Pediatrics and Prevention, CHD and Pediatrics and Quality Improvement, Interventions and Structural Heart Disease, Interventions and Vascular Medicine, Hypertension
Keywords: Aneurysm, Dissecting, Aortic Valve Insufficiency, Cardiac Surgical Procedures, Genetics, Hypertension, Marfan Syndrome, Risk Factors, Secondary Prevention, Smoking Cessation, Vascular Diseases
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