Table 1.
Age (Years) | 53 ± 17 |
Females (n, %) | 19 (79.2) |
WHO FC I/II/III/IV (%) | 0/12.5/62.5/25 |
NTproBNP (pg/mL) | 669 (IQR 153–1938) |
Previous PEA (n, %) | 6 (25) |
Haemodynamics | |
Systolic PAP (mmHg) | 84.5 ± 23.5 |
Mean PAP (mmHg) | 51.3 ± 12.6 |
Mean RAP (mmHg) | 10.7 ± 4.0 |
PCWP (mmHg) | 11.0 ± 2.7 |
CI (L/min/m2) | 2.30 ± 0.64 |
PVR (WU) | 10.66 ± 4.59 |
SaO2 (%) | 89.6 ± 4.6 |
SvO2 (%) | 60.9 ± 8.3 |
Heart Rate | 82 ± 9 |
PH therapy | |
ERA (n, %) | 9 (37.5) |
PDE5-I (n, %) | 1 (4.2) |
sGC stimulator (n, %) | 14 (58.3) |
Oral IP receptor agonist (n, %) | 1 (4.2) |
IV epoprostenol | 1 (4.2) |
Sc treprostinil | 2 (8.3) |
Inhaled Iloprost | 1 (4.2) |
None/single/double/triple (%) | 20.8/45.8/25.0/8.3 |
Home Oxygen (n, %) | 21 (87.5) |
Associated Conditions | |
Splenectomy (n, %) | 6 (25) |
Hemoglobinopathies (n, %) | 4 (16.7) |
Myeloproliferative disorder (n, %) | 2 (8.3) |
Thrombophilic disorder (n, %) | 5 (20.8) |
V/A shunt/Pacemaker (n, %) | 2 (8.3) |
APS (n, %) | 2 (8.3) |
Psychiatric disorder (n, %) | 1 (4.2.) |
WHO FC: World Health Organization Functional Class, PEA: Pulmonary Endarterectomy, PAP: Pulmonary Arterial Pressure, PCWP: Pulmonary Capillary Wedge Pressure, CI: Cardiac Index, PVR: Pulmonary Vascular Resistance, SaO2: Arterial Oxygen Saturation, SvO2: Mixed Venous Oxygen Saturation, PH: Pulmonary Hypertension, ERA: Endothelin Receptor Antagonist, PDE5-I: I: Phosphodiesterase-5 inhibitor, sGC: soluble Guanylate cyclase, IV: intravenous, Sc: Subcutaneous, APS: Antiphospholipid Syndrome, V/A: Ventriculoatrial.