|
Aortic root dilatation
|
-.118
|
-.491–.225
|
.531
|
|
Previous aortic root surgery
|
-.054
|
-.449–.342
|
.787
|
|
Aortic root diameter (cm)
|
.308
|
.011–.604
|
.042
|
|
Z-score of aortic root diameter
|
.035
|
-.051–.122
|
.414
|
|
LV ejection fraction (%)
|
-.021
|
-.036–.006
|
.006
|
|
LVESD (mm/m2)
|
.024
|
. -.018–.065
|
.258
|
|
LVEDD (mm/m2)
|
.043
|
-.007–.079
|
.021
|
|
PASP (mmHg)2
|
.023
|
.001–.045
|
.040
|
|
Family history of Marfan
|
.132
|
-.217–.482
|
.452
|
|
Ectopia lentis
|
-.183
|
-.531–.165
|
.298
|
|
Pneumothorax
|
-.133
|
-.617–.351
|
.585
|
|
Dural ectasia (Habermann)
|
.095
|
-.254–.443
|
.590
|
|
Striae distensae
|
-.021
|
-.331–.373
|
.906
|
|
Myopia > −3 diopters
|
-.294
|
-.691–.102
|
.143
|
|
Mitral valve prolapse
|
.224
|
-.427–-.020
|
.081
|
|
Systemic score ≥ 7 points
|
-.026
|
-.415–.364
|
.895
|
|
- Total systemic score points
|
-.010
|
-.076–.054
|
.764
|
|
- Skeletal score points
|
-.030
|
-.113–.053
|
.470
|
|
- Non-skeletal score points
|
.033
|
-.095–.161
|
.610
|
|
FBN1 mutation characteristics
| | | |
|
PTC mutations
|
-.150
|
-.550–.249
|
.455
|
|
Splicing mutations
|
.208
|
-.422–.837
|
.513
|
|
Any mutation affecting Cys
|
.027
|
-.359–.412
|
.890
|
|
Missense affecting Cys
|
-.026
|
-.536–.484
|
.919
|
|
Any mutation in cbEGF
|
-.133
|
-.365–.262
|
.589
|
|
Missense in cbEGF
|
-.021
|
-.564–.606
|
.943
|
|
Any mutation in LTBP-bd
|
-.540
|
−1.240–.160
|
.128
|
|
Missense mutation in LTBP-bd
|
.018
|
−1.106–1.142
|
.974
|
|
Any mutation in exons 24–32
|
-.135
|
-.643–.373
|
.597
|
|
Missense in exons 24–32
|
-.014
|
-.654–.629
|
.965
|
