Table 1 Creatine transport deficiency subject electrocardiogram (ECG) and echocardiographic summary findings.
QTc < 470 milliseconds | QTc ≥ 470 milliseconds | P value | |
Number of subjects | 11 | 7 | NS |
Age (years) | 8.0 ± 4.4 | 6.5 ± 2.8 | NS |
Weight (kg) | 22.6 ± 9.9 | 17.3 ± 7.7 | NS |
BSA | 0.85 ± .27 | 0.72 ± .21 | NS |
Electrocardiogram findings | |||
Heart rate (bpm) | 97.5 ± 23.3 | 95.9 ± 12.5 | NS |
Rhythm | All NSR | All NSR | NS |
P axis (degrees) | 42.3 ± 19.1 | 38.3 ± 25.0 | NS |
QRS axis (degrees) | 76.0 ± 19.9 | 56.0 ± 37.4 | NS |
T axis (degrees) | 38.6 ± 21.0 | 16.4 ± 15.7 | P = 0.047 |
PR interval (milliseconds) | 121.7 ± 17.2 | 129.1 ± 14.4 | NS |
QRS interval (milliseconds) | 79.6 ± 6.3 | 79.7 ± 9.4 | NS |
QT interval (milliseconds) | 357.8 ± 38.3 | 405.3 ± 43.4 | P = 0.047 |
QTc interval (milliseconds) | 448.3 ± 15.9 | 510.3 ± 29.0 | P < 0.0001 |
T-wave abnormality | 3/11 | 6/7 | P = 0.049 |
Echocardiographic findings | |||
EF (%) | 64.2 ± 2.1 | 65.7 ± 3.5 | NS |
FS (%) | 37.8 ± 2.4 | 39.7 ± 5.0 | NS |
LVIDd (mm) | 33.9 ± 5.2 | 34.7 ± 4.1 | NS |
LVIDd z-score | −0.93 ± 1.0 | 0.22 ± 0.74 | P = 0.0059 |
LViDs (mm) | 20.0 ± 6.7 | 21.1 ± 3.2 | NS |
LViDs z-score | −0.86 ± .75 | −0.32 ± 1.27 | NS |
IVS (mm) | 5.6 ± 0.9 | 5.1 ± 0.7 | NS |
IVS z-score | −1.2 ± 0.5 | −1.3 ± 0.6 | NS |
LVPWDd (mm) | 5.7 ± 0.8 | 5.0 ± 0.6 | P = 0.0183 |
LVPWDd z-score | −0.85 ± .49 | −1.30 ± .35 | NS(.11) |
LV mass (g) | 47.0 ± 17.5 | 42.8 ± 14.9 | NS |
LV mass z-score | −1.5 ± 0.9 | −0.95 ± .76 | NS(.065) |
LV mass indexed | 54.2 ± 10.1 | 57.6 ± 9.5 | NS |
LV mass index z-score | −1.7 ± 0.9 | −1.2 ± 0.7 | P = 0.0417 |
Pro-BNP (pg/mL) (# of subjects with samples) | 147 ± 98.0(5) | 270 ± 102(3) | NS |
