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Table 4 Relationship of variables with MPA diameter in 72 Marfan patients 1

From: The main pulmonary artery in adults: a controlled multicenter study with assessment of echocardiographic reference values, and the frequency of dilatation and aneurysm in Marfan syndrome

Clinical variables Regression coefficient (b) 95% confidence interval P 2
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
  1. cbEGF indetifies calcium-binding epidermal growth factor-like domain; coefficient, regression coefficient; Cys, cysteine; LV, left ventricular; LVEDD, indexed left ventricular end-diastolic diameter, LVESD, indexed left ventricular end-systolic diameter, LTBP-bd, latent TGF-beta-binding domain; missense, missense mutation; N, number; PASP, pulmonary artery systolic pressure; and PTC, premature termination codon. 1We analyzed on those 72 patients, whom we recruited in Hamburg. 2Linear regression analysis. 2PASP measurements were 22 ± 12 mmHg (range 6 – 49 mmHg).