The disease-modifying therapy in idiopathic pulmonary arterial hypertension: clinical case of calcium channel blocker therapy in long-term vasoresponder

Rare vasoreactive test (VRT) performance limits the effective and cheap calcium channel blocker (CCB) therapy in potential long-term vasoresponders patients with idiopathic pulmonary arterial hypertension (PAH). Long-term vasoresponders exhibit the unique (5–7 %) idiopathic PAH population with the highest survival. We presented the clinical case of a young idiopathic PAH with positive VRT and a significant advantage of CCB therapy over PAH-specific therapy. Pulmonary artery pressure and exercise tolerance normalization, complete reverse right heart remodeling were achieved on CCB therapy. Our case emphasized the importance of careful identification of VRT positive idiopathic PAH patients and the meticulous follow-up during CCB treatment.