Table 1

Demographic, clinical and haemodynamic characteristics at presentation to the United Kingdom Service for Pulmonary Hypertension in Children

All patientsIncidentPrevalentp Value
Demographic
Subjects (n)644420
Age (years) at:
 Diagnosis4.3 (1.5–8.9)4.3 (1.5–10.1)4.6 (1.7–8.4)0.74
 Presentation6.5 (2.6-13.0)5.0 (1.6–10.8)10.5 (5.4–14.6)0.01
Female patients n (%)40 (63)27 (61)13 (65)0.78
Clinical
WHO I/II/III/IV0/12/34/180/9/23/120/3/11/60.78
Atrial communication, n (%)28 (44)22 (50)6 (30)0.14
Mean weight z-score−0.66−0.46−1.110.17
Mean height z-score−0.71−0.57−1.010.33
Haemodynamic
Baseline:(n=58)(n=39)(n=19)
RAP (mm Hg)7.1±3.47.4±3.46.3±3.20.32
mPAP (mm Hg)58±2257±2560±140.62
mSAP (mm Hg)63±1062±967±130.11
CI (l/min/m2)2.9±1.12.8±1.13.3±1.00.16
PVRI (U.m2)19.7±13.320.5±14.717.8±9.10.50
Acute vasodilator test:(n=54)(n=37)(n=17)
mPAP (mm Hg)51±2253±2549±170.61
mSAP (mm Hg)63±1161±1167±100.09
PVRI (U.m2)14.7±11.315.5±12.512.6±7.70.41
ARs (n)5230.31
  • Data are presented as mean±SD; except for age, median (IQR), comparisons made using t test, Fisher's exact and Wilcoxon rank-sum tests p value - incident versus prevalent. Boldface highlights the stastically significant p values throughout tables 1 & 2.

  • AR, acute responder; CI, cardiac index; mPAP, mean pulmonary artery pressure; mSAP, mean systemic arterial pressure; PVRI, pulmonary vascular resistance index; RAP, mean right atrial pressure.