Table 1 Baseline clinical and echocardiographic features of the 26 patients with MPS III
From: Cardiac characteristics and natural progression in Taiwanese patients with mucopolysaccharidosis III
No. | Gender | MPS type | 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 |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
1 | M | IIIB | 1.8 | −0.98 | 3.20 | 4.08 | 1.08 | −2.15 | − 1.86 | − 0.48 | − 0.12 | 0.62 | 0.14 | 62% | 39% | – |
2 | M | IIIB | 2.2 | −0.51 | 0.67 | 0.44 | 0.53 | 0.70 | 0.00 | −0.43 | 0.44 | 1.38 | 0.14 | 73% | 41% | – |
3 | F | IIIA | 3.6 | 0.10 | 2.73 | 4.07 | 0.85 | −2.22 | −0.85 | 2.86 | 0.48 | 2.53 | 1.68 | 57% | 28% | – |
4 | M | IIIB | 4.2 | 0.44 | −1.50 | 2.07 | 0.41 | 0.83 | −0.04 | −0.19 | − 0.88 | − 0.53 | − 1.28 | 74% | 42% | – |
5 | M | IIIB | 4.3 | −0.58 | NA | −0.11 | NA | 0.62 | 0.14 | −0.73 | NA | 1.83 | −0.06 | 69% | 38% | – |
6 | F | IIIB | 4.9 | −1.73 | 1.50 | 2.29 | −0.17 | −1.20 | − 0.88 | − 0.85 | − 0.66 | 0.65 | − 0.88 | 57% | 37% | – |
7 | M | IIIB | 5.1 | −2.31 | −0.05 | −1.25 | −0.24 | 0.14 | −2.13 | −0.87 | − 0.25 | 0.71 | − 0.55 | 85% | 53% | – |
8 | M | IIIB | 5.1 | 0.19 | −0.60 | 0.90 | 1.18 | 1.97 | 1.07 | −0.57 | −0.25 | 2.39 | −0.39 | 54% | 38% | – |
9 | F | IIIB | 5.2 | 0.02 | 1.19 | 0.65 | 0.33 | 0.13 | −1.60 | 0.02 | −0.75 | 1.35 | −0.17 | 81% | 48% | – |
10 | F | IIIB | 5.2 | 0.28 | 1.45 | 1.47 | 0.54 | −0.68 | −2.92 | 2.86 | 0.64 | 1.47 | 0.36 | 88% | 57% | – |
11 | M | IIIB | 5.4 | 1.21 | 0.00 | 4.13 | 0.35 | −0.07 | 0.64 | 0.42 | 1.11 | 0.67 | −0.39 | 60% | 31% | – |
12 | F | IIIB | 6.0 | −0.82 | 1.87 | 1.31 | −1.14 | −0.07 | 0.64 | −1.13 | −1.39 | −0.28 | 2.10 | 60% | 31% | – |
13 | M | IIIB | 7.2 | 0.36 | 1.875 | 2.63 | 1.33 | 0.41 | −0.28 | −0.69 | 2.59 | 0.61 | −0.34 | 72% | 41% | – |
14 | M | IIIB | 7.5 | 0.58 | 1.65 | 3.24 | 0.78 | −0.89 | −0.79 | 1.38 | −0.93 | 0.72 | 0.90 | 59% | 38% | – |
15 | M | IIIB | 7.8 | 1.10 | 1.29 | 2.00 | −0.48 | 1.26 | 1.38 | 0.52 | −0.79 | 2.83 | −1.03 | 54% | 34% | – |
16 | M | IIIA | 9.9 | −1.54 | −1.28 | − 1.04 | −0.15 | 1.44 | 0.04 | −1.51 | −0.76 | 1.83 | 1.26 | 74% | 43% | – |
17 | F | IIIB | 10.5 | −0.40 | NA | 1.37 | NA | −0.03 | −0.61 | 0.75 | NA | 1.88 | −0.60 | 72% | 40% | – |
18 | F | IIIA | 11.0 | −1.61 | 0.96 | 2.44 | 0.90 | −1.51 | −0.68 | −0.44 | − 1.26 | 0.56 | − 1.35 | 63% | 33% | – |
19 | F | IIIB | 11.4 | 0.50 | NA | 5.56 | 0.83 | −1.52 | −0.57 | 0.31 | −0.13 | 2.86 | −1.57 | 60% | 31% | – |
20 | F | IIIB | 11.5 | −0.09 | −0.09 | 0.11 | 0.04 | 0.55 | −0.89 | 0.43 | 1.27 | 0.06 | 0.63 | 77% | 45% | – |
21 | F | IIIB | 12.3 | −1.48 | 2.04 | 2.03 | 0.56 | −0.12 | −0.38 | −1.13 | −0.44 | 3.56 | −0.72 | 70% | 39% | – |
22 | F | IIIB | 12.9 | −1.60 | NA | 0.11 | NA | −1.74 | −2.32 | 0.38 | NA | 1.26 | −2.75 | 75% | 43% | – |
23 | M | IIIA | 13.6 | 0.50 | NA | 0.91 | NA | 0.97 | −0.20 | 0.36 | NA | 1.74 | −1.52 | 73% | 42% | – |
24 | M | IIIA | 16.4 | −0.20 | NA | 2.00 | NA | 0.00 | −0.25 | 0.92 | NA | 5.19 | −4.29 | 68% | 38% | – |
25 | F | IIIB | 18.5 | 0.23 | NA | 1.33 | NA | 1.62 | 1.24 | 1.73 | NA | 6.17 | −0.34 | 66% | 36% | + |
26 | M | IIIC | 26.5 | −1.13 | −1.76 | 1.74 | 0.23 | 0.00 | 0.54 | 0.04 | −1.71 | 0.12 | −0.44 | 63% | 33% | – |