Table 1.
Baseline characteristics of patients with and without mitral annular disjunction on their initial echocardiograms
| Variable | Total | MAD | No MAD | P-value |
|---|---|---|---|---|
| n = 185 | n = 110 | n = 75 | ||
| Male, n (%) | 99 (54) | 54 (49) | 45 (60) | 0.144 |
| Age in years (IQR) | 10.2 (5.2, 14.4) | 10.1 (5.2, 14.0) | 10.3 (5.1, 14.8) | 0.739 |
| Race–ethnicity, n (%) | 0.159 | |||
| Non-Hispanic White | 85 (46) | 52 (47) | 33 (44) | |
| Non-Hispanic Black | 18 (10) | 15 (14) | 3 (4) | |
| Hispanic | 60 (32) | 32 (29) | 28 (37) | |
| Asian | 11 (6) | 6 (5) | 5 (7) | |
| Not reported | 11 (6) | 5 (5) | 6 (8) | |
| Weight in kg (IQR) | 39.5 (20.5, 65.8) | 34.3 (20.1, 63.5) | 49.5 (22.2, 70.0) | 0.101 |
| Height in cm (IQR) | 158 (123, 178) | 155 (127, 176) | 154 (120, 177) | 0.499 |
| BSA in m2 (IQR) | 1.34 (0.83, 1.80) | 1.20 (0.86, 1.71) | 1.17 (0.81, 1.78) | 0.171 |
| Blood pressure | ||||
| Systolic in mmHg ± SD | 106 ± 14 | 105 ± 14 | 107 ± 14 | 0.591 |
| Diastolic in mmHg ± SD | 62 ± 9 | 61 ± 9 | 63 ± 8 | 0.221 |
| Documented FBN1 variant, n (%) | 164 (89) | 96 (87) | 68 (91) | 0.991 |
| Ectopia lentis, n (%) | 80 (44) | 45 (42) | 35 (49) | 0.358 |
| MVP, n (%) | 59 (32) | 53 (48) | 6 (8) | <0.001 |
| Bi-leaflet | 42 (23) | 41 (37) | 1 (1) | <0.001 |
| Anterior leaflet only | 7 (4) | 3 (3) | 4 (5) | 0.444 |
| Posterior leaflet only | 10 (5) | 9 (8) | 1 (1) | 0.051 |
| MV regurgitation severity, n (%) | <0.001 | |||
| None–mild | 160 (86) | 87 (79) | 73 (97) | |
| Moderate–severe | 25 (14) | 23 (21) | 2 (3) | |
| LVEF in % (IQR) | 63 (60, 66) | 62 (59, 65) | 64 (60, 67) | 0.024 |
| LVEF < 55% | 5 (3) | 5 (5%) | 0 | 0.082 |
| Aortic root in mm (IQR) | 30.6 (24.7, 35.1) | 31.2 (26.6, 36.2) | 28.0 (23.3, 34.3) | 0.014 |
| Aortic root z-score (IQR) | 2.5 (1.6, 3.9) | 3.1 (2.2, 4.8) | 1.9 (1.2, 2.6) | <0.001 |
| z-score ≥ 2, n (%) | 118 (64) | 84 (76) | 34 (45) | <0.001 |
| Age Quartile 1 (<5.2 years) | 3.1 (2.2, 4.8) | 3.7 (2.9, 5.5) | 2.9 (2.0, 3.5) | 0.043 |
| Age Quartile 2 (5.2–10.2 years) | 2.5 (1.9, 3.9) | 3.1 (2.2, 4.3) | 2.2 (1.2, 2.5) | 0.006 |
| Age Quartile 3 (10.2–14.4 years) | 2.4 (1.3, 3.8) | 3.3 (2.2, 4.9) | 1.4 (0.7, 2.0) | <0.001 |
| Age Quartile 4 (14.4–21.0 years) | 2.0 (1.1, 2.8) | 2.6 (1.3, 4.3) | 1.8 (1.0, 2.2) | 0.013 |
BSA, body surface area; IQR, inter-quartile range; LVEF, left ventricular ejection fraction; MAD, mitral annular disjunction; MV, mitral valve; MVP, mitral valve prolapse.