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Table 1 Baseline clinical, echocardiographic and electrocardiographic features of 32 patients with MPS IVA

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

No. Gender Age (years) LVMI (z score) RVDd (z score) IVSd (z score) IVSs (z score) LVIDd (z score) LVIDs (z score) LVPWd (z score) LVPWs (z score) AoD (z score) LAD (z score) EF SF (%) Reversed E/A ratio MS MR AS AR MVP Thick IVS Electrocardiographic features
1 M 1.1 0.40 -0.07 1.83 -0.32 -0.18 -0.70 1.09 -1.22 0.52 -0.86 0.74 42% - 0 1 0 0 + - Normal
2 M 1.3 -1.83 1.56 -0.16 -1.03 1.08 1.85 -1.61 -0.17 1.36 -0.10 0.60 31% - 0 0 0 0 - - Sinus tachycardia
3 M 2.0 -0.11 2.18 2.25 0.34 -0.32 -1.55 -0.25 0.26 3.56 0.14 0.80 47% - 0 0 0 0 - - NA
4 F 3.4 0.33 3.18 1.28 0.36 0.46 -0.58 -0.07 -1.36 1.71 -1.40 0.76 43% - 0 0 0 0 - - Sinus tachycardia, RAD
5 F 3.7 -1.40 0.31 0.20 -0.23 -0.19 -0.60 -0.97 -1.06 1.31 1.36 0.74 41% - 0 0 0 0 - - Normal
6 M 5.3 0.69 2.71 0.99 0.29 1.22 0.05 0.21 2.00 3.00 1.64 0.76 43% - 1 0 1 0 - - Sinus tachycardia, RAD
7 M 5.8 1.34 0.50 2.24 -0.03 0.14 0.49 1.73 -2.64 1.64 -1.30 0.65 34% - 0 0 0 0 - - Sinus tachycardia, septal hypertrophy
8 M 6.0 -0.43 0.98 0.14 -1.19 1.64 1.15 -0.71 -0.92 5.26 -0.05 0.71 40% - 0 0 0 0 - - LAD
9 F 6.4 1.13 0.59 3.77 -0.52 -0.90 -0.26 1.60 -1.33 1.73 0.26 0.66 35% - 0 0 0 0 - - Sinus arrhythmia
10 F 6.5 0.42 3.73 2.48 1.55 0.37 0.15 0.16 0.64 3.06 -0.24 0.69 38% - 1 1 0 0 + - Normal
11 M 6.5 0.36 4.59 4.89 2.13 -1.00 -0.09 -0.07 -2.24 3.86 0.30 0.62 32% - 0 0 0 0 - - RAD
12 F 8.0 0.17 3.50 0.45 0.03 0.83 0.31 -0.28 -0.82 3.94 1.12 0.71 40% - 1 0 1 0 - - Sinus arrhythmia, low voltage
13 M 8.9 2.61 NA 5.47 1.56 1.43 -0.36 -0.19 1.52 4.00 4.80 0.79 46% - 1 1.5 1 0 - + Sinus arrhythmia
14 M 9.0 -0.82 0.45 3.00 0.79 -1.29 -1.00 1.51 -0.82 2.18 1.88 0.69 37% + 0 0 0 0 - + Sinus tachycardia, LAD
15 F 10.7 -0.72 0.59 3.51 1.05 -2.46 -2.92 1.06 -1.71 1.82 1.44 0.80 47% - 1 0 1 0 + + Sinus tachycardia
16 M 10.7 1.62 -0.84 0.37 2.65 4.20 2.13 -1.45 -0.12 4.47 -0.21 0.76 45% - 1 0 0 0 - + Sinus arrhythmia
17 F 10.8 -1.12 3.11 0.39 -0.46 -0.06 -0.08 -0.59 -0.14 0.94 -1.00 0.68 37% - 0 0 0 0 - - Normal
18 F 11.1 -2.08 1.30 0.37 -1.39 -0.79 0.50 -0.75 -2.01 1.39 0.71 0.55 28% - 0 0 0 0 - - Normal
19 F 11.5 -2.52 2.23 0.21 -0.27 -1.46 -2.40 -0.29 -0.32 1.36 -1.30 0.80 47% - 0 0 0 0 - - Sinus arrhythmia
20 F 13.3 0.61 0.41 0.68 0.57 0.40 -0.19 0.86 0.48 4.53 1.20 0.73 41% - 1 0 1 0 - - Sinus arrhythmia
21 F 13.6 -0.14 2.45 1.73 1.45 -0.50 -0.26 0.59 0.16 3.47 0.26 0.69 38% - 1 1 1 0 + - Sinus arrhythmia
22 F 15.1 1.08 0.60 2.50 -0.46 0.48 -0.44 -0.18 0.76 4.18 0.70 0.74 42% - 0 0 1 0 + - Sinus tachycardia
23 F 15.3 2.01 NA 2.45 1.94 2.32 0.16 -0.18 -0.58 4.71 0.03 0.78 46% - 0 0 0 0 - + Sinus arrhythmia
24 M 15.7 0.45 0.73 1.73 1.45 1.54 0.04 0.37 0.41 3.64 -0.75 0.77 44% - 0 0 0 0 - - NA
25 M 15.8 5.49 -0.13 4.63 6.26 4.03 1.08 1.84 0.76 4.65 -0.10 0.79 47% - 0 0 0 0 - - NA
26 F 15.8 3.36 2.23 6.60 1.84 0.67 -0.21 1.37 1.67 6.40 0.34 0.76 43% + 1 1 1 1 + + Sinus tachycardia, LAE
27 M 15.9 1.58 -0.32 1.60 0.88 3.20 1.75 -0.22 0.13 2.48 1.59 0.68 39% - 0 0 0 0 - - NA
28 F 16.1 -0.72 1.20 1.14 -1.47 -0.27 -0.29 -0.75 -1.45 3.76 -0.68 0.67 37% - 0 0 0 0 - - Sinus tachycardia
29 F 18.4 5.14 -2.50 15.07 5.20 -0.95 -0.65 4.71 1.62 2.82 0.04 0.68 36% - 0 0.5 1 0 - + NA
30 M 21.3 2.08 -2.68 2.13 4.67 2.15 -0.96 -0.24 0.69 6.47 -1.16 0.85 54% + 1 0 1 0 + + Sinus tachycardia, RAE
31 M 25.3 3.43 -0.13 4.51 2.29 1.02 -0.61 0.65 2.37 3.44 -1.90 0.78 46% - 1 0 1 1.5 - + Sinus arrhythmia, IRBBB
32 M 29.1 7.65 3.57 8.00 2.84 1.03 -0.60 3.39 2.41 6.53 0.17 0.78 46% + 1 1 2 2 - + Normal
  1. MPS mucopolysaccharidosis, LVMI left ventricular mass index, 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, SF shortening fraction, E/A ratio between early and late (atrial) ventricular filling velocity, MS mitral stenosis, MR mitral regurgitation, AS aortic stenosis, AR aortic regurgitation, MVP mitral valve prolapse, IVS interventricular septum, NA not available, RAD right axis deviation, LAD left axis deviation, LAE left atrial enlargement, RAE right atrial enlargement, IRBBB irregular bundle branch block. Severity of valvular stenosis and regurgitation (MS, MR, AS, AR) were estimated and graded on the following scores: 0 (none), 1 (mild), 2 (moderate), and 3 (severe)