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. 2022 Apr 7;12(2):e12055. doi: 10.1002/pul2.12055

Table 1.

Summary of key consensus opinions on how to escalate therapy for PAH patients failing to achieve goals of therapy

Patient on dual therapy
  • Switching within a class or to a new class could be considered if tolerance, side effects, logistics, patient preference, and so on, are impacting therapy
  • Switching from a PDE5i to an sGC activator could be considered for patients failing to improve and/or with dose‐limiting side effects from the PDE5i and at low‐to‐moderate risk
  • Adding an agent from a new class should be considered for patients whose disease is progressing or who are not reaching target goals
    • o
      For patients who are rapidly progressing, in a high‐risk group, or in WHO FC IV, the added agent should be a parenteral PCA
Patient on triple oral therapy
  • Dose adjustment could be considered if side effects are not significant and progression is not rapid, based on hemodynamics and risk
  • Switching from a PDE5i to an sGC activator could be considered if the patient is not improving and all medications are at the maximum‐tolerated dose, particularly in patients with good adherence and mild symptom worsening, or if the patient is unable or unwilling to escalate to parenteral PCAsa
  • Escalating from oral to parenteral PCAs should be considered for patients who are in FC IV, are having rapid progression, have high‐risk hemodynamics, are at maximum‐tolerated doses of all PH meds, or have right heart failure, and for moderate‐risk patients with clinical worsening
Patient on triple therapy including an SQ/IV PCA
  • Dose adjustment could be considered if the patient is not at the maximally tolerated dose for all three medications
  • Switching within the class could be considered for intolerability and adherence issues
  • Switching from a PDE5i to an sGC activator could be considered if the patient is not improving or all medications are at the maximum‐tolerated dosea
  • Switching from SQ/IV treprostinil to IV epoprostenol could be considered in very sick patients on a maximum‐tolerated dose of treprostinil
  • Referral for lung transplant evaluation in appropriate candidates

Abbreviations: FC, functional class; PAH, pulmonary arterial hypertension; PCA, prostacyclin analog; PDE5i, phosphodiesterase‐5 inhibitor; sGC, soluble guanylate cyclase; SQ/IV, oral or infusion; WHO, World Health Organization.

a

This switch has not been studied in patients on triple oral therapy.