PURPOSE: Bicuspid aortic valve (BAV) is associated with increased prevalence of annulo-aortic ectasia, dissection and ascending aortic aneurysm. This study was undertaken to compare the amount of fibrillin-1 and elastin in the media of great vessels of patients with bicuspid and tricuspid aortic valve disease. METHOD: Tissue samples of ascending aorta and pulmonary artery were obtained from 22 patients with bicuspid aortic valve disease (BAV) and 17 patients with tricuspid aortic valve disease (TAV), including 6 normal valves from the transplant program. Indirect immunofluorescence and computerized image analyses were used to quantify fibrillin-1 and elastin of the media of the arteries. The results were expressed as the mean integrated optical density (IOD). RESULTS: In the ascending aorta, the IOD for fibrillin-1 was 15 ± 8 in the BAV group and 24 ± 7 in the TAV group (p = 0.001). In the pulmonary artery, the IOD for fibrillin-1 was 18 ± 10 in the BAV group and 25 ± 9 in the TAV group (p = 0.07). In respect to the elastin measurements of the aorta, the specific IOD was 34 ± 13 in the BAV group and 29 ± 14 in the TAV group. In the pulmonary artery the IOD for elastin was 30 ± 12 in the BAV group and 29 ± 14 in the TAV group (p = 0.34). CONCLUSIONS: Patients with BAV were found to have less fibrillin-1 in the ascending aorta and pulmonary artery than patients with TAV. These findings may explain aortic root dilation and ascending aortic dissection in patients with BAV disease.
Aortic valve; Aorta; Aorta; Aorta; Fibrillin; Elastin; Marfan syndrome
