Table 1

Patient demographics for patients with IPAH/HPAH and APAH

ParameterIPAH/HPAH (n=59)APAH (n=71)P value
Female, n (%)26 (44%)43 (60%)0.062
Age, years10.20±6.347.77±6.330.033
Weight, kg33.75±24.2729.47±21.140.286
Height, cm123.85±37.85116.88±37.490.298
Body surface area, m21.04±0.520.946±0.490.307
Treatment at RHC, n (%)
No treatment16 (27%)27 (38%)0.191
Monotherapy by oral route9 (15%)15 (13%)0.394
Double therapy by oral route19 (36%)20 (28%)0.621
Combination therapy using prostacyclin analogue15 (25%)9 (13%)0.062
Vasoreactivity, n (%)15 (25%)7 (9%)0.018
mRAP, mm Hg5.48±2.696.71±3.290.022
mPAP, mm Hg68.43±41.2782.54±44.220.087
Systolic RVP, mm Hg62.56±11.4858.94±12.200.082
PVRi, Pa s/m³7.21±7.938.13±7.010.489
PCWP, mm Hg7.01±3.179.08±3.280.0004
RVCI, L/min/m23.56±0.974.12±1.650.045
Heart rate, bpm85±1998±260.0016
Ees/Ea_(Kind)1.51±0.491.50±0.520.993
Ees/Ea_(Takeuchi)0.92±0.68095±0.630.754
  • Data are expressed as mean values (SD), n (%), unless otherwise noted.

  • Bold p-values indicate significance.

  • APAH, associated pulmonary arterial hypertension; Ees/Ea_(Kind), vascular–ventricular coupling ratio Kind method; Ees/Ea_(Takeuchi), vascular–ventricular coupling Takeuchi method; IPAH/HPAH, idiopathic and hereditary pulmonary arterial hypertension; mPAP, mean pulmonary arterial pressure; mRAP, mean right atrial pressure; PCWP, pulmonary capillary wedge pressure; PVRi, indexed pulmonary vascular resistance; RHC, right heart catheterisation; RVCI, right ventricular cardiac index; RVP, right ventricular pressure.