Table 4.
Prevalence of echocardiographic abnormalities at the last available echocardiogram, including both ERT treated and untreated subjects.
| Overall (n = 76) | MPS I (n = 27) | MPS II (n = 22) | MPS IV (n = 19) | MPS VI (n = 8) | p-value | |
|---|---|---|---|---|---|---|
| LVM Z score > 2 | 6/76 (8%) | 2/27 (7%) | 2/22(9%) | 2/19 (11%) | 0/8 (0%) | 0.821 |
| RWT > 0.41 | 19/76 (25%) | 7/27 (26%) | 7/22 (32%) | 3/19 (16%) | 2/8 (25%) | 0.701 |
| Concentric remodeling | 16/76 (21%) | 5/27 (19%) | 6/22 (27%) | 3/19 (16%) | 2/8 (25%) | 0.697 |
| Concentric hypertrophy | 3/76 (4%) | 0/27 (0%) | 1/22 (5%) | 2/19 (11%) | 0/8 (0%) | |
| Eccentric hypertrophy | 3/76 (4%) | 2/27 (7%) | 1/22 (5%) | 0/19 (0%) | 0/8 (0%) | |
| LAD Z score > 2 | 20/76 (26%) | 7/27 (26%) | 4/22 (18%) | 7/19 (37%) | 2/8 (25%) | 0.605 |
| SPAP > 35 mmHg* | 11/40 (28%) | 6/14 (43%) | 2/12 (17%) | 1/9 (11%) | 2/5 (40%) | 0.265 |
| LVEF <55% | 1/76 (1%) | 0/27 (0%) | 0/22 (0%) | 1/19 (5%) | 0/8 (0%) | 0.385 |
LAD, Left atrial diameter; LVEF, Left ventricle ejection fraction; LVM, left ventricle mass; RWT, relative wall thickness; SPAP, systolic pulmonary artery pressure.
*
SPAP was measured in 40 of the total 76 available last echocardiograms of the participants. Statistical analysis with chi-squared.