TABLE 1

Patient characteristics and resting haemodynamic and pulmonary function

Female/male16/10
Age years45 (30–52)
BMI kg·m−225.7±5.0
Pulmonary hypertension aetiology
 PAH (idiopathic/heritable/HIV-associated)17 (10/5/2)
 CTEPH9
Newly diagnosed cases9
Duration of prevalent PH years4.4 (1.6–9.7)
Haemodynamics
 Right atrial pressure mmHg7±3
 Mean pulmonary artery pressure mmHg49±14
 Pulmonary artery wedge pressure mmHg9±3
 Cardiac output L·min−15.7±1.8
 Cardiac index L·min−1·m−23.2±0.9
 Pulmonary vascular resistance Wood units7±3
 Mixed venous oxygen saturation %67±8
PAH-targeted therapy
 None10
 Calcium channel blockers2
 Oral monotherapy3
 Dual oral combination therapy3
 PDE-5 inhibitor + i.v. epoprostenol1
 Triple combination therapy + i.v. epoprostenol7
NYHA functional class I/II/III/IV10/9/7/0
6MWD m517±101
Lung function test
 FEV1/VC %82±10
 FEV1 % pred96±18
 TLC % pred94±16
DLCO % pred65±15
KCO % pred76±20
PaO2 mmHg80±13
PaCO2 mmHg33±4

Data are presented as n, median (interquartile range) or mean±sd. BMI: body mass index; PAH: pulmonary arterial hypertension; CTEPH: chronic thromboembolic pulmonary hypertension; PH: pulmonary hypertension; PDE: phosphodiesterase; NYHA: New York Heart Association; 6MWD: 6-min walk distance; FEV1: forced expiratory volume in 1 s; VC: vital capacity; TLC: total lung capacity; DLCO: diffusing capacity of the lung for carbon monoxide; KCO: carbon monoxide transfer coefficient; PaO2: arterial partial pressure of oxygen; PaCO2: arterial partial pressure of carbon dioxide.