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

From: Cardiac features and effects of enzyme replacement therapy in Taiwanese patients with Mucopolysaccharidosis IVA

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 (normal1) 1.41 ± 0.27 (n=12) 1.16 ± 0.26 (n=14) 0.025
TV E/A (normal1) 1.30 ± 0.36 (n=11) 1.31 ± 0.32 (n=10) 0.928
  1. 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