IPAH or what else? – IPAH with pulmonary comorbidity

Pulmonary hypertension is divided into 5 distinct groups, with group 1 reflecting pulmonary arterial hypertension (PAH). The PH-specific drug therapies approved to date interfere with three different signaling pathways (NO signaling pathway, prostacyclin signaling pathway, and endothelin receptor signaling pathway). Much has happened since the first PAH-specific drug (bosentan) was approved in Europe about 20 years ago. Numerous other agents have been added and have improved the prognosis of PAH patients.