Table 3– Clinical and haemodynamic data at the time of diagnosis in pulmonary arterial hypertension (PAH) subtypes and chronic thromboembolic pulmonary hypertension (CTEPH)
PAHCTEPHANOVA p-value
IPAHCTDCHDHIVPoPHTOSPVOD
Subjects n (%)314 (30)157 (15)167 (16)54 (5)61 (6)33 (3.2)15 (1.5)162 (16)
Age yrs46±1854±15 *36±17*39±5*52±10*39±13*51±1561±15*<0.001
Males %2710*2742*49312240*<0.001
6MWD m382±117309±115*365±101416±126396±109372±139314±127317±112*<0.001
WHO FC %
 I–II2921384053132223<0.001
 III5964544046724468
 IV1115819216339
Haemodynamics
 Mean Ppa mmHg55±1547±13*68±19*52±1149±13*67±17*55±1147±13*<0.001
Pra mmHg8±59±6*9±68±68±58±59±48±50.577
 Cardiac index L·min−1·m−22.4±0.72.6±0.93.0±0.9*2.4±0.63.2±0.9*2.2±0.52.2±0.52.3±0.5<0.001
 PVR Wood units12±611±6*13±712±67±3*17±8*13±610±5*<0.001
Sv,O2 %62±959±1166±10*60±1069±7*59±857±1761±10<0.001
  • Data are presented as mean±sd, unless otherwise stated. IPAH: idiopathic PAH; CTD: connective tissue disease; CHD: congenital heart disease; PoPH: portopulmonary hypertension; TOS: toxic oil syndrome; PVOD: veno-occlusive disease; 6MWD: 6-min walk distance; WHO: World Health Organization; FC: functional class; Ppa: pulmonary artery pressure; Pra: right atrial pressure; PVR: pulmonary vascular resistance; Sv,O2: mixed-venous oxygen saturation. *: p<0.05 compared with IPAH.