Table 1.
Characteristics of patients with MFS and LDS
| Total (n = 168) | MFS (n = 103) | LDS (n = 65) | P-value | |
|---|---|---|---|---|
| Clinical characteristics | ||||
| Age at first exam ination (years) | 24 ± 17 | 20 ± 14 | 32 ± 20 | <0.001 |
| Male sex, n (%) | 90 (54) | 56 (54) | 34 (52) | 0.79 |
| Family members, n (%) | 90 (54) | 44 (43) | 46 (71) | <0.001 |
| Echocardiographic parameters | ||||
| Age at echocardiography (years) | 33 ± 17 | 31 ± 15 | 36 ± 19 | 0.04 |
| Aortic root dilatation Z-score (n = 131, pre-event echocardiographies) | 4.1 ± 2.6 | 4.6 ± 2.6 | 3.4 ± 2.5 | 0.009 |
| MAD, n (%) | 69 (41) | 47 (46) | 22 (34) | 0.13 |
| Posterolateral MAD distance (mm) | 8 ± 3 | 9 ± 3 | 7 ± 3 | 0.05 |
| Posterolateral MAD distance indexed (mm) | 4.6 ± 1.7 | 4.9 ± 1.9 | 3.9 ± 1.2 | 0.03 |
| Anterolateral MAD distance (mm) | 7 ± 3 | 7 ± 2 | 6 ± 4 | 0.16 |
| Anterolateral MAD distance indexed (mm) | 4.0 ± 2.1 | 4.2 ± 2.1 | 3.3 ± 2.1 | 0.22 |
| MVP, n (%) | 52 (31) | 41 (40) | 11 (17) | 0.002 |
| Mitral regurgitation, n (%) | 30 (18) | 22 (21) | 8 (13) | 0.15 |
| Diastolic mitral annulus diameter indexed (mm/m2) | 18 ± 5 | 18 ± 4 | 18 ± 5 | 0.98 |
| Systolic mitral annulus diameter indexed (mm/m2) | 17 ± 5 | 18 ± 5 | 16 ± 5 | 0.06 |
| Dilated mitral annulus (n = 136, adult patients only), n (%) | 46 (27) | 35 (34) | 11 (17) | 0.005 |
| LVEDVi (mL/m2) | 57 ± 19 | 59 ± 17 | 54 ± 21 | 0.10 |
| LVESVi (mL/m2) | 23 ± 9 | 24 ± 9 | 21 ± 9 | 0.05 |
| EF (%) | 60 ± 5 | 60 ± 5 | 62 ± 5 | 0.03 |
| Outcome data | ||||
| AoE, n (%) | 112 (67) | 66 (64) | 46 (71) | 0.37 |
| Aortic dissection, n (%) | 27 (16) | 15 (15) | 12 (18) | 0.50 |
| Prophylactic aortic surgery, n (%) | 85 (51) | 51 (50) | 34 (52) | 0.72 |
| Age at AoE (years) | 35 ± 14 | 33 ± 13 | 39 ± 16 | 0.01 |
| Aortic root dilatation Z-score at prophylactic surgery on ascending aorta (n = 81) | 6.6 ± 3.2 | 7.6 ± 3.4 | 5.0 ± 2.3 | <0.001 |
| Mitral events, n (%) | 12 (7) | 7 (7) | 5 (8) | 0.81 |
| Age at mitral event (years) | 37 ± 15 | 31 ± 15 | 45 ± 11 | 0.12 |
Values are average ± SD or frequencies(%). P-values are calculated by Student’s t test or chi-square, as appropriate. Bold values denote statistical significance.
AoE, aortic events; EF, ejection fraction; LDS, Loeys–Dietz syndrome; LVEDVi, left ventricular end-diastolic volume indexed for body surface area; LVESVi, left ventricular end-systolic volume indexed for body surface area; MAD, mitral annulus disjunction; MFS, Marfan syndrome; MVP, mitral valve prolapse.