Phenotypes and treatment outcomes in idiopathic pulmonary arterial hypertension (IPAH) patients with comorbidities

IPAH is often diagnosed in elderly patients with frequent comorbidities. Whereas a clear treatment strategy and risk assessment is recommended for patients with rare classical IPAH, monotherapy with phos­phodi­esterase type 5 inhibitors (PDE5i) or endothelin receptor antagonists (ERA) followed by regular follow-up and individualized therapy should be used for frequent patients with cardiopulmonary comorbidities. Here, we focus on these patients with IPAH and comorbidities, present a review of the literature with a focus on recently published work, and summarize factors that may help to provide guidance for individualized treatment approaches in such patients.