Table 1.
Characteristics of the study population
| mean ± SD or n (%) | ||||||
|---|---|---|---|---|---|---|
| Demographics | Age | (years) | 53 | ± | 14.65 | |
| BMI | (kg/m 2 ) | 27.9 | ± | 5.69 | ||
| Gender | male | n (%) | 21 | (38.9) | ||
| female | n (%) | 33 | (61.1) | |||
| Diagnosis | IPAH | n (%) | 31 | (57.4) | ||
| HPAH | n (%) | 8 | (14.8) | |||
| APAH | n (%) | 12 | (22.2) | |||
| CTEPH | n (%) | 3 | (5.6) | |||
| WHO functional class | I | n (%) | 1 | (1.9) | ||
| II | n (%) | 36 | (66.7) | |||
| III | n (%) | 17 | (31.5) | |||
| PAH-targeted medication | Endothelin receptor antagonist | 40 | (74.1) | |||
| Phosphodiesterase-5-inhibitors | 38 | (70.4) | ||||
| Soluble guanylate cyclase-stimulator | 8 | (13.0) | ||||
| Prostanoids | 6 | (14.8) | ||||
| Calcium channel blockers | 2 | (03.7) | ||||
| Combination therapy | ||||||
| Mono | n (%) | 18 | (33.3) | |||
| Double | n (%) | 31 | (57.4) | |||
| Triple | n (%) | 5 | (9.3) | |||
| RHC | Rest | mPAP | (mmHg) | 35.5 | ± | 11.69 |
| sPAP | (mmHg) | 57.6 | ± | 20.87 | ||
| dPAP | (mmHg) | 23 | ± | 7.87 | ||
| PCWP | (mmHg) | 10 | ± | 3.54 | ||
| PVR | (dyn*sec*cm −5 ) | 393.4 | ± | 235.03 | ||
| CO | (l/min) | 5.8 | ± | 1.61 | ||
| CI | (l/min/m 2 ) | 3 | ± | 0.73 | ||
| SVI | (ml/m 2 ) | 41.1 | ± | 10.2 | ||
| 25 W | ∆ CI | (l/min/m 2 ) | 1.2 | ± | 0.67 | |
| 50 W | ∆ CI | (l/min/m 2 ) | 2 | ± | 0.93 | |
| 75 W | ∆ CI | (l/min/m 2 ) | 2.6 | ± | 1.15 | |
| Peak | mPAP | (mmHg) | 56.5 | ± | 15.91 | |
| sPAP | (mmHg) | 90.2 | ± | 28.36 | ||
| dPAP | (mmHg) | 36.1 | ± | 11.56 | ||
| CO | (l/min) | 10.2 | ± | 3.49 | ||
| CI | (l/min/m 2 ) | 5.3 | ± | 1.59 | ||
| SVI | (ml/m 2 ) | 47.2 | ± | 13.9 | ||
| Echocardiography | RV area | (cm 2 ) | 20.1 | ± | 5.59 | |
| RA area | (cm 2 ) | 16.8 | ± | 6.62 | ||
| TAPSE | (cm) | 2.3 | ± | 0.38 | ||
| Cardiopulmonary exercise testing (CPET) | peak V’O2 | (ml/min) | 1126 | ± | 428.88 | |
| peak V’O2/kg | (ml/min/kg) | 14.1 | ± | 3.92 | ||
| sPAP Max | (mmHg) | 81.8 | ± | 27.87 | ||
| 6-MWD | (m) | 423 | ± | 113.09 | ||
| Laboratory analysis | NT-proBNP | (pg/ml) | 470.3 | ± | 856.74 | |
| Pulmonary function test (PFT) | DLCOc SB | (% Soll) | 58.6 | ± | 17.25 | |
| DLCOc VA | (% Soll) | 70.42 | ± | 20.00 | ||
IPAH = idiopathic pulmonary arterial hypertension, HPAH = heritable PAH, APAH = associated PAH, CTEPH = chronic thromboembolic PH, RHC = right heart catheter, BMI = Body Mass Index, RV = right ventricular, RA = right atrial, TAPSE = tricuspid annular plane systolic excursion, VO’2 = oxygen consumption, NT-proBNP = N-terminal pro brain natriuretic peptide, DLCOc SB = diffusing capacity transfer factor, DLCOc / VA = diffusing capacity transfer coefficient, mPAP = mean pulmonary arterial pressure, sPAP = systolic PAP, dPAP = diastolic PAP, PCWP = pulmonary capillary wedge pressure, PVR = pulmonary vascular resistance, CI = Cardiac Index, SVI = stroke volume index, HR = heart rate, SV = stroke volume, ∆ = difference