Table 2:
Cumulative Number of Cardiovascular Outcomes in the GenTAC Cohort
| Outcome | Total N=2,686 |
MFS N=861 |
BAV N=879 |
LDS N=121 |
nsHTAD N=378 |
TS N=298 |
vEDS N=149 |
p-value a | |
|---|---|---|---|---|---|---|---|---|---|
| Elective proximal aortic aneurysm surgery, ever had, n (%) | No | 2,035 (75.8) | 588 (68.3) | 625 (71.1) | 86 (71.1) | 296 (78.3) | 291 (97.7) | 149 (100.0) | <0.01 |
| Yes | 651 (24.2) | 273 (31.7) | 254 (28.9) | 35 (28.9) | 82 (21.7) | 7 (2.3) | 0 (0.0) | ||
|
| |||||||||
| Any aortic dissection surgery, ever had, n (%) | No | 2,441 (90.9) | 749 (87.0) | 846 (96.2) | 105 (86.8) | 302 (79.9) | 293 (98.3) | 146 (98.0) | <0.01 |
| Yes | 245 (9.1) | 112 (13.0) | 33 (3.8) | 16 (13.2) | 76 (20.1) | 5 (1.7) | 3 (2.0) | ||
|
| |||||||||
| DeBakey dissection type, n (%)n | Type I | 88 (36.4) | 36 (32.7) | 12 (36.4) | 8 (50.0) | 30 (40.0) | 1 (20.0) | 1 (33.3) | <0.01 |
| Type II | 65 (26.4) | 18 (15.5) | 19 (57.6) | 3 (18.8) | 21 (28.0) | 4 (80.0) | 0 (0.0) | ||
| Type IIIa | 33 (13.2) | 21 (18.2) | 1 (3.0) | 2 (12.5) | 9 (12.0) | 0 (0.0) | 0 (0.0) | ||
| Type IIIb | 56 (22.7) | 34 (30.9) | 1 (3.0) | 3 (18.8) | 16 (20.0) | 0 (0.0) | 2 (66.7) | ||
|
| |||||||||
| Mortality, n (%) | CVM | 56 (2.1) | 27 (3.1) | 10 (1.1) | 2 (1.7) | 9 (2.4) | 2 (0.7) | 6 (4.0) | <0.01 |
| Other causes | 33 (1.2) | 14 (1.6) | 4 (0.5) | 0 (0.0) | 12 (3.2) | 1 (0.3) | 2 (1.3) | ||
Diagnosis abbreviations: MFS, Marfan syndrome; BAV, Bicuspid aortic valve with enlargement; LDS, Loeys Dietz syndrome; nsHTAD, Non-syndromic Heritable thoracic aortic disease; TS, Turner syndrome; vEDS, vascular Ehlers Danlos syndrome
Abbreviations: CVM, cardiovascular mortality; SD, standard deviation
a
p-values from chi-square tests
b
n=3 dissections could not be classified