Definitions of acute and long-term response
| Acute pulmonary vasoreactivity# for patients with idiopathic, hereditable or drug-induced PAH | Reduction of mPAP ≥10 mmHg to reach an absolute value of mPAP ≤40 mmHg |
| Increased or unchanged cardiac output | |
| Long-term response to CCBs | New York Heart Association Functional Class I/II |
| With sustained haemodynamic improvement (same or better than achieved in the acute test) after at least 1 year on CCBs only |
PAH: pulmonary arterial hypertension; mPAP: mean pulmonary arterial pressure; CCB: calcium channel blocker. #: nitric oxide (10–20 ppm) is recommended for performing vasoreactivity testing, but i.v. epoprostenol, i.v. adenosine or inhaled iloprost can be used as alternatives.