Gaps in evidence in the treatment of prevalent patients with pulmonary arterial hypertension at intermediate risk: An expert consensus

Vascul Pharmacol. 2024 Dec:157:107432. doi: 10.1016/j.vph.2024.107432. Epub 2024 Sep 10.

Abstract

Despite the innovations introduced in the 2022 European Society of Cardiology/European Respiratory Society Guidelines on Pulmonary Hypertension, risk discrimination and management of pulmonary arterial hypertension (PAH) patients at intermediate risk still represents a grey zone. Additionally, clinical evidence derived from currently available studies is limited. This expert panel survey intends to aid physicians in choosing the best therapeutic strategy for patients at intermediate risk despite ongoing oral therapy. An expert panel of 24 physicians, specialized in cardiology and/or pulmonology with expertise in handling all drugs available for the treatment of PAH participated in the survey. All potential therapeutic options for patients at intermediate risk were explored and analyzed to produce graded consensus statements regarding: the switch from endothelin receptor antagonist (ERA) or phosphodiesterase 5 inhibitor (PDE5i) to another oral drug of the same class; the addition of a drug targeting the prostacyclin pathway administered by different routes; the switch from PDE5i to riociguat.

Keywords: Expert consensus; Intermediate risk; PAH therapeutic options; Pulmonary arterial hypertension.

Publication types

  • Review
  • Practice Guideline

MeSH terms

  • Antihypertensive Agents* / adverse effects
  • Antihypertensive Agents* / therapeutic use
  • Arterial Pressure / drug effects
  • Clinical Decision-Making
  • Consensus*
  • Endothelin Receptor Antagonists* / adverse effects
  • Endothelin Receptor Antagonists* / therapeutic use
  • Evidence-Based Medicine
  • Humans
  • Phosphodiesterase 5 Inhibitors* / adverse effects
  • Phosphodiesterase 5 Inhibitors* / therapeutic use
  • Prevalence
  • Pulmonary Arterial Hypertension* / diagnosis
  • Pulmonary Arterial Hypertension* / drug therapy
  • Pulmonary Arterial Hypertension* / epidemiology
  • Pulmonary Arterial Hypertension* / physiopathology
  • Pulmonary Artery / drug effects
  • Pulmonary Artery / metabolism
  • Pulmonary Artery / physiopathology
  • Risk Assessment
  • Risk Factors
  • Treatment Outcome

Substances

  • Antihypertensive Agents
  • Phosphodiesterase 5 Inhibitors
  • Endothelin Receptor Antagonists