Table 5.
Age at diagnosis relative to age at cardiovascular event
| MFS | BAV | LDS | nsHTAD | TS | vEDS | p-value a | |
|---|---|---|---|---|---|---|---|
| Mean age at diagnosis, mean (SD) | 15.7 (14.8) | 37.0 (21.0) | 18.7 (15.9) | 35.8 (18.9) | 6.1 (7.7) | 25.7 (15.7) | <0.001 |
| Difference between age in years at diagnosis and age at elective proximal aortic aneurysm surgery, median (IQR) b | 12.0 (2.0–20.0) | 1.0 (1.0–6.0) | 1.0 (−0.5–1.0) | 1.0 (1.0–6.0) | 21.5 (14.5–28.5) | - | <0.001 |
| Difference between age in years at diagnosis and age at any aortic dissection surgery, median (IQR) b | 13.0 (1.0–23.0) | 1.0 (0.0–4.0) | 1.0 (0.0–2.5) | 0.0 (0.0–1.0) | 30.0 (-) | 1.0 (−1.0–2.0) | <0.001 |
| Difference between age in years at diagnosis and age at emergent proximal dissection surgery, median (IQR) b | 2.0 (0.0–18.0) | 1.0 (0.0–1.0) | 1.0 (−3.0–1.0) | 0.0 (0.0–1.0) | 30.0 (-) | -1.0 (-) | <0.001 |
| Difference in age in years between diagnosis and CVM, median (IQR) b | 14.5 (10.0–22.0) | 4.5 (2.0–11.0) | 22.0 (-) | 14.0 (8.5–17.5) | 28.0 (-) | 5.5 (3.0–7.0) | 0.037 |
Diagnosis abbreviations: MFS, Marfan syndrome; BAV, Bicuspid aortic valve with enlargement; LDS, Loeys Dietz syndrome; nsHTAD, Non-syndromic HTAD, Heritable thoracic aortic disease; TS, Turner syndrome; vEDS, vascular Ehlers Danlos syndrome
Abbreviations: CVM, cardiovascular-related mortality; IQR, interquartile range; SD, standard deviation
a
p-values from t-tests for normally distributed variables and Mann-Whitney U tests for non-normally distributed data
b
Positive differences indicate a diagnosis occurred prior to an event and negative differences indicate a diagnosis occurred after an event