Before treatment initiation | After treatment initiation | Treatment-induced change | p-value¶ | |
Clinical diagnostics | ||||
Heart rate beats per min | 97±23.3 (n = 50) | 92±24.0 (n = 50) | −5±18.4 (n = 50) | 0.090 |
Systolic blood pressure mmHg | 104±16.4 (n = 44) | 97±10.8 (n = 44) | −7±12.6 (n = 44) | 0.001 |
Diastolic blood pressure mmHg | 63±10.7 (n = 41) | 59±7.8 (n = 41) | −3±12.2 (n = 41) | 0.104 |
WHO-FC | ||||
I or II | 11/55 (20.0) | 19/55 (34.5) | −0.3±0.66 | 0.003 |
III | 29/55 (52.7) | 29/55 (52.7) | ||
IV | 15/55 (27.3) | 7/55 (12.7) | ||
6MWD+ m | 349±97.5 (n = 28) | 371±97.1 (n = 28) | 22±57.0 (n = 28) | 0.050 |
Biochemical characteristics | ||||
NT-proBNP ng·L−1 | 666 (96–1543) (n = 36) | 243 (101–686) (n = 36) | −54 (−673–31) (n = 36) | 0.011 |
CK U·L−1 | 67 (33−106) (n = 23) | 83 (56−112) (n = 23) | 13 (−13–40) (n = 23) | 0.066 |
CK-MB U·L−1 | 21 (9–33) (n = 23) | 19 (11–29) (n = 23) | −2 (−10–4) (n = 23) | 0.242 |
Uric acid mmol·L−1 | 0.3±0.07 (n = 33) | 0.3±0.10 (n = 33) | 0.0±0.07 (n = 33) | 0.695 |
Noradrenaline nmol·L−1 | 1.5 (0.85–1.99) (n = 16) | 1.9 (1.25–2.90) (n = 16) | 0.4 (−0.62–1.00) (n = 16) | 0.179 |
Adrenaline nmol·L−1 | 0.2 (0.13–0.75) (n = 16) | 0.3 (0.20–0.39) (n = 16) | 0.1 (−0.13–0.15) (n = 16) | 0.698 |
Detectable troponin | 6/26 (23.1) | 6/26 (23.1) | 6/26 (23.1) | >0.999 |
Echocardiographic characteristics | ||||
TAPSE mm | 15 (11.8–17.0) (n = 41) | 16 (13.0–17.8) (n = 41) | 1 (−1.0–2.6) (n = 41) | 0.077 |
RV/LV ratio | 0.96 (0.69–1.47) (n = 40) | 0.84 (0.67–1.20) (n = 40) | −0.06 (−0.23–0.05) (n = 40) | 0.029 |
Presence of pericardial effusion | 4/42 (9.5) | 5/42 (11.9) | 1/42 (2.4) | >0.999 |
Data are presented as mean±sd, n/N (%) or median (interquartile range), unless otherwise stated. WHO-FC: World Health Organization functional class; 6MWD: 6-min walk distance; NT-proBNP: N-terminal pro-brain natriuretic peptide; CK: creatine kinase; TAPSE: tricuspid annular plane systolic excursion; RV: right ventricular; LV: left ventricular. #: this table encompasses only patients who survived until the first follow-up (n = 57); ¶: comparison between before and after treatment initiation, with paired-samples t-test, Wilcoxon signed rank test or McNemar test where appropriate; +: only children ≥7 years old.