Enalapril and Metoprolol 1) | Placebo 1) | |||
---|---|---|---|---|
Age [years] | 12 ± 1.2 | n = 21 | 11 ± 1.1 | n = 17 |
Body Mass Index [kg/m2] | 23 ± 6 | n = 20 | 21 ± 5 | n = 17 |
Ability to rise from supine position | 24% | 5/21 | 24% | 4/17 |
Preserved ability to walk | 33% | 7/21 | 41% | 5/17 |
Maximum walking distance [m] | 200 (3–6000)2) | n = 7 | 300 (30–800)2) | n = 5 |
Systolic blood pressure [mmHg] | 103 ± 16 | n = 21 | 101 ± 11 | n = 17 |
Patients with presence of cardiac symptoms | 0% | 0/21 | 0% | 0/17 |
Steroid use or history of steroid use | 76% | 16/21 | 59% | 10/17 |
NYHA class | ||||
Not applicable | 52% | 11/21 | 59% | 10/17 |
NYHA class I | 48% | 10/21 | 35% | 6/17 |
NYHA class II | 0% | 0/21 | 6% | 1/17 |
Quality of life (KINDL total score at screening) | 70.8 ± 10.1 | n = 21 | 75.6 ± 10.0 | n = 17 |
Echocardiography | n = 21 | n = 17 | ||
Left ventricle fractional shortening [%] | 35 ± 3 | 36 ± 4 | ||
Electrocardiogram (ECG) | n = 21 | n = 17 | ||
Ventricular heart rate [beats/min] | 84 ± 14 | 94 ± 16 | ||
Holter-Electrocardiogram (Holter-ECG) | n = 20 | n = 15 | ||
Ventricular heart rate [beats/min] | ||||
minimum | 67 ± 12 | 72 ± 10 | ||
maximum | 126 ± 17 | 133 ± 11 | ||
mean | 90 ± 12 | 97 ± 9 | ||
Heart rate variability (Holter ECG) | n = 11 | n = 10 | ||
Mean NN [ms] | 678 ± 80 | 571 ± 174 | ||
SDNN [ms] | 98 ± 29 | 96 ± 30 | ||
SDANN [ms] | 71 ± 24 | 75 ± 25 | ||
ASDNN [ms] | 60 ± 20 | 54 ± 21 | ||
rMSSD [ms] | 46 ± 26 | 45 ± 21 | ||
pNN50 [%] | 17 ± 15 | 13 ± 11 |