Table 1.

Patient Characteristics

	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).