Table 1

Baseline characteristics and haemodynamics of patients with PAH–CHD

VariableOverall groupEisenmenger syndrome with pre-tricuspid defectEisenmenger syndrome with post-tricuspid defectPAH with systemic-to-pulmonary shuntPAH with small defectsPAH after defect correction
Patients, n (%)32 (100)7 (22)15 (47)1 (3)2 (6)7 (22)
BNP (pg/mL)3044±63326983±13 7502020±24841124204±1072643±2849
6-MWD (m)321±116344±143305±107300427±23310±135
WHO FC IV17(53)3 (43)10(67)01 (50)3 (42)
mRAP (mm Hg)11±67±313±699±312±6
mPAP (mm Hg)73±2168±1280±194550±372±34
PVR (WU)19±1016±622±103011±420±4
CI (L/min/m2)2.1±0.62.4±0.72.1±0.62.42±0.21.9±0.2
SpO2 (%)86±888±781±79495±0.793±2
  • All data are presented as means±SD.

  • 6-MWD, 6 min walking distance; BNP, B-type brain natriuretic peptide; CHD, congenital heart disease; CI, cardiac index; mPAP, mean pulmonary artery pressure; mRAP, mean right atrial pressure; PAH, pulmonary arterial hypertension; PVR, pulmonary vascular resistance; SpO2, systemic oxygen saturation; WHO FC, WHO functional class; WU, Wood units.