In this matched‐pair analysis of patients with newly diagnosed PAH receiving monotherapy or combination therapy as initial treatment, we found more pronounced improvements in WHO FC, BNP/NT‐proBNP and risk status in patients treated with combination therapy at first follow up, while there were no significant differences in 6MWD, while no difference could be seen in up to 5 years survival, hospitalisation and continuation rates of PAH treatment between the two groups based on initial therapy strategy only (not taking into account possible therapy escalation). The gene discussed is NPPB; the disease is pulmonary arterial hypertension.