Characteristics | All patients# | IPAH | All patients with APAH¶ | APAH subgroups+ | |||
CHD | CVD/CTD | Portal hypertension | Drugs/ toxins | ||||
Subjects n | 2525 | 1166 | 1280 | 250 | 639 | 136 | 134 |
P̄pa mmHg | 50.7±13.6 | 52.1±13.0 | 49.1±13.8 | 59.5±16.9 | 44.9±11.2 | 48.5±10.6 | 52.2±12.2 |
Subjects n | 2525 | 1166 | 1280 | 250 | 639 | 136 | 134 |
p-value | <0.001ƒ | ||||||
Ppcw mmHg | 9.1±3.5 | 9.2±3.5 | 9.0±3.5 | 8.9±3.6 | 8.9±3.5 | 9.3±3.6 | 9.2±3.6 |
Subjects n | 2525 | 1166 | 1280 | 250 | 639 | 136 | 134 |
p-value | 0.14 | ||||||
P̄ra mmHg | 9.3±5.6 | 9.9±5.7 | 8.6±5.5 | 7.2±4.5 | 8.7±5.6 | 8.3±5.9 | 10.7±5.9 |
Subjects n | 2298 | 1050 | 1174 | 229 | 580 | 131 | 127 |
p-value | <0.001ƒ | ||||||
PVRI Wood units·m2 | 21.1±12.5 | 22.9±11.4 | 19.0±13.0 | 23.7±20.9 | 16.9±9.1 | 15.7±7.2 | 24.2±12.7 |
Subjects n | 1868 | 842 | 965 | 186 | 488 | 100 | 100 |
p-value | <0.001ƒ | ||||||
Fick or thermodilution CI L·min−1·m2§ | 2.4±0.8 | 2.2±0.8 | 2.5±0.9 | 2.7±1.0 | 2.5±0.8 | 2.8±0.8 | 2.1±0.8 |
Subjects n | 1868 | 842 | 965 | 186 | 488 | 100 | 100 |
p-value | <0.001ƒ | ||||||
SV,O2 % | 62.9±10.0 | 61.8±9.8 | 64.2±10.1 | 67.3±9.5 | 63.3±10.0 | 66.6±8.5 | 61.1±9.7 |
Subjects n | 1456 | 665 | 738 | 148 | 356 | 86 | 96 |
p-value | <0.001ƒ |
Data are presented as mean±sd, unless otherwise stated. PAH: pulmonary arterial hypertension; IPAH: idiopathic PAH; APAH: associated PAH; CHD: congenital heart disease; CVD/CTD: collagen vascular disease/connective tissue disease; P̄pa: mean pulmonary artery pressure; Ppcw: pulmonary capillary wedge pressure; P̄ra: mean right atrial pressure; PVRI: pulmonary vascular resistance index; CI: cardiac index; SV,O2: mixed venous oxygen saturation. #: all patients aged ≥19 yrs at diagnosis with a Ppcw ≤15 mmHg enrolled during the consecutive screening of newly and previously diagnosed patients, including those with WHO group I diagnoses other than IPAH or APAH (i.e. familial PAH, pulmonary veno-occulsive disease and persistent pulmonary hypertension of the newborn); ¶: all APAH patients, including those with associated PAH subgroups other than CHD, CVD/CTD, portal hypertension, and drugs/toxins (i.e. HIV and others); +: APAH subgroups are mutually exclusive according to the following hierarchy for patients with multiple associated-PAH diagnoses: CHD, CVD/CTD, portal hypertension, drugs/toxins, HIV and others; §: the Fick CI is used unless it is missing, in which case thermodilution CI is used; ƒ: p-value for all haemodynamic parameters is obtained from the two-sample t-test examining the difference in the distribution of the characteristics among patients diagnosed with IPAH versus all patients with APAH. Reproduced from [15] with permission from the publisher.