Effects of pulmonary arterial hypertension-targeted therapy on World Health Organization (WHO)/New York Heart Association (NYHA) functional class, exercise capacity and haemodynamics at first follow-up visit in patients with severe sarcoidosis-associated pulmonary hypertension#

BaselineFirst follow-up visitDifferencep-value
WHO/NYHA functional class I–II/III/IV11/52/1826/45/100.01
6MWD m311±127324±138+13 m0.33
RAP mmHg7±46±4−14%0.007
mPAP mmHg48±942±11−13%<0.00001
Cardiac index L·min−1·m−12.6±0.82.9±0.8+12%<0.00001
PVR Wood units9.7±4.46.9±3.0−29%<0.00001

Data are expressed as n or mean±sd, unless otherwise stated. 6MWD: 6-min walk distance; RAP: right atrial pressure; mPAP: mean pulmonary artery pressure; PVR: pulmonary vascular resistance. #: n=81; : median (interquartile range) 4.5 (4.0–6.7) months.