|
Echocardiographic parameter
|
Without valvular thickening (n=18)
|
With valvular thickening (n=14)
|
p value
|
|---|
|
Gender (male/female)
|
10/8
|
6/8
|
0.492
|
|
Age (years)
|
8.7 ± 5.4
|
14.4 ± 7.1
|
0.015
|
|
Height (z score)
|
-5.00 ± 3.20
|
-8.92 ± 3.21
|
0.002
|
|
Weight (z score)
|
-1.83 ± 1.13
|
-2.63 ± 0.93
|
0.042
|
|
BMI (z score)
|
0.02 ± 1.06
|
0.53 ± 1.24
|
0.219
|
|
BSA (m2)
|
0.70 ± 0.25
|
0.70 ± 0.08
|
0.956
|
|
LVMI (g/m2.7)
|
53.9 ± 24.1
|
80.9 ± 46.6
|
0.042
|
|
LVMI (z score)
|
0.11 ± 1.86
|
2.00 ± 2.30
|
0.015
|
| SF (%) (normal ≧28) |
39.3 ± 6.0
|
43.2 ± 4.6
|
0.051
|
|
RVDd (z score)
|
1.32 ± 1.35 (n=17)
|
1.05 ± 2.20 (n=13)
|
0.683
|
|
IVSd (z score)
|
1.78 ± 1.54
|
3.89 ± 3.99
|
0.047
|
|
IVSs (z score)
|
0.40 ± 1.81
|
1.82 ± 1.63
|
0.029
|
|
LVIDd (z score)
|
0.44 ± 1.56
|
0.71 ± 1.51
|
0.633
|
|
LVIDs (z score)
|
-0.03 ± 1.08
|
-0.33 ± 1.06
|
0.441
|
|
LVPWd (z score)
|
0.09 ± 1.03
|
0.76 ± 1.58
|
0.159
|
|
LVPWs (z score)
|
-0.82 ± 0.95
|
0.83 ± 1.20
|
0.0001
|
|
AoD (z score)
|
2.56 ± 1.47
|
4.15 ± 1.44
|
0.005
|
|
LAD (z score)
|
-0.07 ± 0.99
|
0.58 ± 1.56
|
0.158
|
|
EF
|
0.70 ± 0.07
|
0.75 ± 0.05
|
0.052
|
|
With diastolic dysfunction (MV E/A<1)
|
n=1 (6%)
|
n=3 (21%)
|
0.189
|
| MV E/A (normal≧1) |
1.41 ± 0.27 (n=12)
|
1.16 ± 0.26 (n=14)
|
0.025
|
| TV E/A (normal≧1) |
1.30 ± 0.36 (n=11)
|
1.31 ± 0.32 (n=10)
|
0.928
|
- MPS mucopolysaccharidosis, BMI body mass index, BSA body surface area, LVMI left ventricular mass index, SF shortening fraction, RVDd right ventricular end diastolic dimension, IVSd interventricular septum thickness in diastole, IVSs interventricular septum thickness in systole, LVIDd left ventricular internal diameter in diastole, LVIDs left ventricular internal diameter in systole, LVPWd left ventricular posterior wall thickness in diastole, LVPWs left ventricular posterior wall thickness in systole, AoD aortic diameter, LAD left atrial dimension, EF ejection fraction, MV mitral valve, E/A ratio between early and late (atrial) ventricular filling velocity, TV tricuspid valve
