Table 1. Characteristics of Patients.
| Baseline characteristic | Total (N = 142) | Patients without MAD (n = 94) | Patients with MAD (n = 48) | P value |
|---|---|---|---|---|
| Age at first examination, median (IQR), y | 25 (12-35) | 28 (15-37) | 16 (8-32) | .005 |
| Pediatric patients, No. (%) | 48 (34) | 24 (26) | 24 (50) | .004 |
| Female, No. (%) | 72 (51) | 52 (55) | 20 (42) | .12 |
| Proband, No. (%) | 70 (49) | 43 (46) | 27 (56) | .24 |
| Baseline MAD distance, median (IQR), mm | 6 (4-12) | NA | 6 (4-12) | NA |
| Bicuspid aortic valve, No. (%) | 6 (4) | 4 (4) | 2 (4) | >.99 |
| Prophylactic aortic surgery prior to inclusion, No. (%) | 10 (7) | 6 (6) | 4 (8) | .73 |
| Aortic dissection prior to inclusion, No. (%) | 5 (4) | 4 (4) | 1 (2) | .66 |
| Type A dissection | 4 (3) | 3 (3) | 1 (2) | >.99 |
| Type B dissection | 1 (1) | 1 (1) | 0 | >.99 |
| Clinical evolution of the population | ||||
| Age at most recent examination, median (IQR), y | 35 (23-49) | 38 (26-50) | 27 (18-48) | .009 |
| Follow-up duration, median (IQR), y | 12.0 (6.2-15.4) | 11.9 (6.0-14.9) | 12.1 (6.5-15.5) | .71 |
| Prophylactic aortic surgery during follow-up, No. (%) | 30/27 (24) | 16/84 (19) | 14/43 (33) | .09 |
| Height, median (IQR), cm | 182 (174-191) | 183 (174-192) | 180 (174-188) | .30 |
| Weight, mean (SD), kg | 74 (21) | 79 (21) | 63 (18) | <.001 |
| BSA, median (IQR), m2 | 1.9 (1.7-2.1) | 2 (1.8-2.2) | 1.8 (1.6-1.9) | <.001 |
| Antihypertensive medication, No. (%) | ||||
| None | 31 (22) | 23 (25) | 8 (17) | .29 |
| β-Blocker | 100 (70) | 66 (70) | 34 (71) | .93 |
| ARB | 30 (21) | 19 (20) | 11 (23) | .71 |
| Other | 7 (5) | 5 (5) | 2 (4) | >.99 |
| Received at least one 24-h Holter monitoring, No. (%) | 103 (73) | 70 (75) | 33 (69) | .47 |
| Echocardiographic data (most recent or prior to surgery) | ||||
| LA diameter, median (IQR), mm | 34 (30-39) | 33 (30-37) | 34 (30-40) | .45 |
| LVESDi, median (IQR), mm/m2 | 17 (14-19) | 16 (14-17) | 19 (17-22) | <.001a |
| LVEDDi, median (IQR), mm/m2 | 25 (23-28) | 24 (22-27) | 29 (25-31) | <.001a |
| LVEF, median (IQR), % | 69 (63-74) | 69 (64-75) | 66 (60-71) | .03a |
| LVEF <55%, No. (%) | 10 (7) | 4 (4) | 6 (13) | .09 |
| MVP, No. (%) | 48 (34) | 14 (15) | 34 (71) | <.001 |
| Bileaflet MVP, No. (%) | 35 (25) | 7 (7) | 28 (58) | <.001 |
| MR severity, No. (%) | ||||
| None | 62 (44) | 52 (55) | 10 (21) | <.001 |
| Grade I or II | 71 (50) | 42 (45) | 29 (60) | |
| Grade III or IV | 9 (6) | 0 | 9 (19) | |
| Mitral annular diameter, mean (SD) | ||||
| End-systolic, mm | 34 (6) | 32 (5) | 38 (6) | <.001 |
| End-diastolic, mm | 32 (6) | 31 (5) | 34 (6) | .007 |
| Aortic root, median (IQR), mmb,c | 42 (37-46) | 41 (36-46) | 42 (37-48) | .34 |
| Aortic root z score, median (IQR)b | 3.3 (2.2-4.3) | 3.0 (1.8-4.0) | 4.1 (2.8-5.7) | <.001 |
| Aortic root z score >3, No. (%)b | 71/127 (56) | 41/84 (49) | 30/43 (70) | .02 |
| AR severity, No. (%)b | ||||
| None | 88/127 (69) | 59/84 (70) | 29/43 (67) | .47 |
| Grade I or II | 34/127 (27) | 23/84 (27) | 11/43 (26) | |
| Grade III or IV | 5/127 (4) | 2/84 (2) | 3/43 (7) |
Abbreviations: AR, aortic regurgitation; ARB, angiotensin receptor blocker; BSA, body surface area; IQR, interquartile range; LA, left atrium; LVEDDi, left ventricular end-diastolic diameter indexed; LVEF, left ventricular ejection fraction; LVESDi, left ventricular end-systolic diameter indexed; MAD, mitral annular disjunction; MR, mitral regurgitation; MVP, mitral valve prolapse; NA, not applicable.
a
Corrected for the presence of aortic and mitral valve regurgitation.
b
Excluding 15 patients with aortic events prior to inclusion.
c
Measured at the aortic sinus.