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Table 1 Baseline characteristics

From: Altered aortic 3D hemodynamics and geometry in pediatric Marfan syndrome patients

 

Marfan

Healthy controls

p-value

N (females)

25 (11)

21 (12)

0.375

Age (y)

15.6 ± 4.0

16.0 ± 2.6

0.720

Height (cm)

178.6 ± 17.3

161.2 ± 11.2

<0.001*

Weight (kg)

65.6 ± 20.2

58.8 ± 15.1

0.208

BSA (m2)

1.78 ± 0.36

1.61 ± 0.25

0.082

BMI (kg/m2)

20.1 ± 4.5

22.4 ± 4.0

0.081

LV ejection fraction (%)

55.7 ± 6.5

58.1 ± 3.7

0.243

Bilateral ectopia lentis (n, %)

5 (20%)

0

 

Genetic mutation (n)

 FBN1 gene

22

-

 

 Unknown

3

-

 

Cardiac medication, (n, %)

20 (80%)

0

 

 1. Beta-blocker

15 (60%)

-

 

 2. AT II-receptor antagonist

7 (28%)

-

 

 3. ACE inhibitor

3 (12%)

-

 

 4. Combination 1 + 2

4 (16%)

-

 

 5. Combination 1 + 3

1 (4%)

-

 

Thoracic aortic size (Z-score)

 Aortic root

3.56 ± 1.45

0.49 ± 0.78

<0.001*

 ST-junction

1.70 ± 1.28

−0.34 ± 0.77

<0.001*

 Ascending aorta

0.21 ± 0.95

−0.54 ± 0.64

0.004*

 Distal aortic arch

0.14 ± 0.90

−0.23 ± 0.66

0.155

 Aortic isthmus

0.96 ± 0.94

0.43 ± 0.49

0.039*

 Proximal descending aorta

2.02 ± 1.60

0.56 ± 0.66

<0.001*

  1. The p value stems from non-paired t-test, for sex from Chi-square test and for thoracic aorta dimensions from the Mann Whitney test, *p < 0.05. Abbreviations: ACE angiotensin converting enzyme, AT angiotensin, BMI body mass index, BSA body surface area, FBN1 fibrillin-1, LV left ventricular, ST sinotubular