ERJ Open Research (Jan 2024)

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

  • Dirk Skowasch,
  • Hans Klose,
  • Ralf Ewert,
  • Heinrike Wilkens,
  • Manuel Richter,
  • Stephan Rosenkranz,
  • Gesine Setzer,
  • Ekkehard Grünig,
  • Michael Halank

DOI
https://doi.org/10.1183/23120541.00668-2023
Journal volume & issue
Vol. 10, no. 1

Abstract

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Idiopathic pulmonary arterial hypertension (IPAH) is often diagnosed in elderly patients with many comorbidities. Whereas a clear treatment strategy and risk assessment is recommended for patients with rare classical IPAH, monotherapy with phosphodiesterase type 5 inhibitors or endothelin receptor antagonists followed by regular follow-up and individualised therapy should be used for patients with many 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 summarise factors that may help to provide guidance for individualised treatment approaches in such patients.