Pulmonary arterial hypertension (PAH) is a disease characterised by a gradual increase in resistance of pulmonary circulation leading to right ventricular failure and death. In only 10 % of cases, there is a response to acute vasoreactivity testing with a significant reduction in mean pulmonary artery pressure (PAP), while in this group of patients, less than one half of cases benefit from long-term treatment with calcium channel blockers (CCB). This paper describes a case report of a young patient with dyspnoea and suspicion of pulmonary hypertension who was referred to a specialised centre. The complex evaluation of her clinical state led to confirmed diagnosis of idiopathic pulmonary arterial hypertension (IPAH). Because there was a positive response to vasoreactivity testing, the treatment for IPAH was initiated with a high dose of CCB. This treatment markedly improved her clinical state as well as echocardiographic and hemodynamic findings.In this study, the authors present a diagnostic algorithm in pulmonary hypertension and emphasise the role of CCB in treatment of PAH in carefully selected patients (Tab. 1, Fig. 5, Ref. 7).