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Published on: December 11, 2017
Incidence of aortic complications in patients with bicuspid aortic valves
Hector I Michelena1, Amber D Khanna, Douglas Mahoney
1Division of Cardiovascular Disease, Mayo Clinic, 200 First St SW, Rochester, MN 55905, USA. michelena.hector@mayo.edu
Insights
Patients with bicuspid aortic valve (BAV) have a low incidence of aortic dissection, but it is significantly higher than in the general population. Long-term data show an increased risk of aortic aneurysm and surgery in BAV patients.
Area of Science:
- Cardiology
- Genetics
- Public Health
Background:
- Bicuspid aortic valve (BAV) is the most common congenital heart defect.
- Long-term, population-based data on aortic complications in BAV patients are limited.
Purpose of the Study:
- To determine the incidence of aortic complications in patients with BAV.
- To compare these incidences with the general population.
Main Methods:
- Retrospective cohort study of Olmsted County residents diagnosed with BAV (1980-1999).
- Long-term follow-up (until 2008-2009) assessing thoracic aortic dissection, ascending aortic aneurysm, and aortic surgery.
- Inclusion of patients with diagnosed and undiagnosed BAV.
Main Results:
- Aortic dissection incidence in BAV patients was 3.1 per 10,000 patient-years, 8.4 times higher than the general population.
- Incidence of ascending aortic aneurysms was 84.9 per 10,000 patient-years, 86.2 times higher than the general population.
- The 25-year rate of aortic surgery was 25% in BAV patients.
Conclusions:
- The incidence of aortic dissection in BAV patients is low but significantly elevated compared to the general population.
- BAV patients face a substantially increased risk of developing aortic aneurysms and requiring aortic surgery.
Context:
Bicuspid aortic valve (BAV), the most common congenital heart defect, has been thought to cause frequent and severe aortic complications; however, long-term, population-based data are lacking.
Objective:
To determine the incidence of aortic complications in patients with BAV in a community cohort and in the general population.
Design, Setting, And Participants:
In this retrospective cohort study, we conducted comprehensive assessment of aortic complications of patients with BAV living in a population-based setting in Olmsted County, Minnesota. We analyzed long-term follow-up of a cohort of all Olmsted County residents diagnosed with definite BAV by echocardiography from 1980 to 1999 and searched for aortic complications of patients whose bicuspid valves had gone undiagnosed. The last year of follow-up was 2008-2009.
Main Outcome Measure:
Thoracic aortic dissection, ascending aortic aneurysm, and aortic surgery.
Results:
The cohort included 416 consecutive patients with definite BAV diagnosed by echocardiography, mean (SD) follow-up of 16 (7) years (6530 patient-years). Aortic dissection occurred in 2 of 416 patients; incidence of 3.1 (95% CI, 0.5-9.5) cases per 10,000 patient-years, age-adjusted relative-risk 8.4 (95% CI, 2.1-33.5; P = .003) compared with the county's general population. Aortic dissection incidences for patients 50 years or older at baseline and bearers of aortic aneurysms at baseline were 17.4 (95% CI, 2.9-53.6) and 44.9 (95% CI, 7.5-138.5) cases per 10,000 patient-years, respectively. Comprehensive search for aortic dissections in undiagnosed bicuspid valves revealed 2 additional patients, allowing estimation of aortic dissection incidence in bicuspid valve patients irrespective of diagnosis status (1.5; 95% CI, 0.4-3.8 cases per 10,000 patient-years), which was similar to the diagnosed cohort. Of 384 patients without baseline aneurysms, 49 developed aneurysms at follow-up, incidence of 84.9 (95% CI, 63.3-110.9) cases per 10,000 patient-years and an age-adjusted relative risk 86.2 (95% CI, 65.1-114; P <.001 compared with the general population). The 25-year rate of aortic surgery was 25% (95% CI, 17.2%-32.8%).
Conclusions:
In the population of patients with BAV, the incidence of aortic dissection over a mean of 16 years of follow-up was low but significantly higher than in the general population.
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