Table 1.
Baseline characteristics
| 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* |
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