Table 4 Biometric characteristics and echocardiographic assessments of the 32 patients with MPS IVA with and without valvular thickening

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/m^2.7)

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