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 | Â |
Genetic mutation (n) | |||
 FBN1 gene | 22 | - |  |
 Unknown | 3 | - |  |
Cardiac medication, (n, %) | 20 (80%) | 0 | Â |
 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* |