Characteristic | All patients |
Subjects | 26 |
Age yrs# | 31±5 |
NYHA FC# | |
I/II | 16 (62) |
III | 8 (31) |
IV | 2 (7) |
6MWD m¶ | 472±97 |
Type of PAH | |
Idiopathic | 17 (64) |
Connective tissue disease | 4 (16) |
Congenital heart disease | 1 (4) |
HIV infection associated | 3 (12) |
Portopulmonary | 1 (4) |
Haemodynamics¶ | |
Pra mmHg | 6±4 |
mPAP mmHg | 44±19 |
Ppcw mmHg | 6±2 |
CI L·min−1·m−2 | 3.1±0.9 |
PVR dyn·s−1·cm−5 | 741±571 |
Sv,O2% | 66±11 |
PAH medication# | |
Oral anticoagulants | 13 |
Calcium channel blockers (responders) | 8 |
Intravenous epoprostenol | 5 |
Subcutaneous treprostinil | 1 |
Inhaled iloprost | 2 |
Endothelin receptor antagonists | 7 |
PDE-5 inhibitors | 7 |
No PAH medication | 4+ |
Data are presented as n, mean±sd or n (%). NYHA FC: New York Heart Association functional class; 6MWD: 6-min walk distance; Pra: right arterial pressure; mPAP: mean pulmonary artery pressure; Ppcw: pulmonary capillary wedge pressure; CI: cardiac index; PVR: pulmonary vascular resistance; Sv,O2: mixed venous oxygen saturation; PDE: phosphodiesterase. #: at the time pregnancy was discovered; ¶: most recent variables at the time pregnancy was discovered; +: in these patients the diagnosis of PAH was made during pregnancy.