Age at the genetic inquest (years) | 44.9 ± 14.2 | |
Male sex (n, %) | 92 | 69.7% |
AAD first event (n, %) | 39 | 29.5% |
Age at AAD (years) | 43.4 ± 11.2 | |
AAD type: Stanford A/B | 36/3 | |
Planned TAA first procedure (n, %) | 43 | 32.6% |
Age at planned TAA first procedure (years) | 39.4 ± 14.2 | |
TAA with no criteria for surgery | 50 | 37.9% |
Age (years) | 45.5 ± 16.0 | |
Dilation of the aortic root, z score ≥ 2 | 43 | 32.6% |
Aortic root dimension (mm, z score) | 44.6 ± 4.5 | 3.9 ± 1.3 |
Dilation of the ascending aorta normalized to BSA > 2 | 27 | 20.5% |
Ascending aorta dimension (mm, normalized to BSA) | 46.3 ± 3.7 | 2.4 ± 0.2 |
Dilation of both aortic root and ascending aorta | 20 | 15.2% |
Age at last clinical clinical examination (years) | 47.3 ± 13.8 | |
Syndromic or familial disease | ||
MFS | 24 | 18.2% |
MFS-like | 15 | 11.4% |
Familial TAAD (n, %) | 45 | 34.1% |
AAD in first-degree relative | 19 | 14.4% |
Unexplained sudden death in first-degree relative | 10 | 7.6% |
Associated structural and functional cardiac abnormalities | ||
BAV | 28 | 21.2% |
Aortic regurgitation | 35 | 26.5% |
Aortic stenosis | 8 | 6.1% |
CoA and PDA | 1 | 0.8% |
MVP with MR (mild/moderate/severe) | 9 (2/5/2) | 6.8% |
Left ventricular noncompaction | 1 | 0.8% |
Hypertrophic cardiomyopathy | 2 | 1.5% |
Other cardiovascular diseases | ||
Hypertension | 73 | 55.3% |
Coronary artery disease | 20 | 15.2% |
Stroke | 8 | 6.1% |
Peripheral artery aneurysms | 6 | 4.5% |
Co-existent abdominal aortic aneurysm | 2 | 1.5% |
Other diseases | ||
Obesity | 20 | 15.2% |
Diabetes mellitus | 10 | 7.6% |
Dyslipidemia | 37 | 28.0% |
Rheumatoid arthritis | 2 | 1.5% |