Table 1.
Affected Individuals (n = 43)∗ | |
---|---|
Sex, M/F | 23/20 |
Age, yrs | 34 (3–74) |
Age at death, yrs | 56 (40–80) |
Age at dissection, yrs | 47.5 (30–80) |
Cardiovascular findings | |
Type A dissections, age, yrs | 4 (51; 40–80) |
Type B dissections, age, yrs | 6 (44.5; 30–57) |
Aortic aneurysm†, age, yrs | 6 (34; 3–68) |
Abdominal aortic surgery‡ | 2 |
Disease beyond aorta§ | 3 |
Skeletal findings | |
Tall stature | 12 |
Arachnodactyly | 16 |
Pectus deformity | 8 |
Kyphoscoliosis | 11 |
Joint hypermobility | 9 |
Loeys-Dietz features | |
Hypertelorism | 14 |
Bifid uvula | 11 |
Cleft palate | 5 |
Values are n, median (range), or n (median; range).
Not all patients were evaluated for all features.
Four aneurysms affected the sinuses of Valsalva, and 2 only affected the ascending aorta.
Surgery was performed on 1 patient at age 43 years and 1 at 50 years.
Cerebral, iliac, or subclavian arteries (n = 1 for each location).