Fig. 1.

Patient case illustrating the benefits of initial combination therapy for PAH. A 34-year-old female presented with evidence of severe pulmonary hypertension. After being diagnosed with PAH, the patient received initial triple combination therapy with a PDE-5i, an ERA and an intravenous PGI₂ analog using a staggered approach to treatment initiation. Significant functional and symptomatic improvements were reported within 2 months following diagnosis. 6MWD 6-min walk distance, ERA endothelin receptor antagonist, FC functional class, I.v. intravenous, PAH pulmonary arterial hypertension, PDE-5i phosphodiesterase type 5 inhibitor, PGI₂ prostacyclin, RHC right heart catheterization. Asterisk indicates an intravenous PGI₂ analog was initially up-titrated to 8 ng/kg/min in hospital and then further up-titrated by 1 ng/kg/min per week at home until a target dose of 20–25 ng/kg/min was reached. For this patient, an oral therapy targeting the PGI₂ pathway could be an alternative option to an intravenous therapy