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PAH Treatment Escalation — EECC MCQ

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HardPulmonary Vascular DiseasePAH Treatment EscalationEECC

A 38-year-old woman with idiopathic PAH (IPAH) classified as intermediate risk on the ESC/ERS risk assessment is started on initial oral combination therapy with ambrisentan and tadalafil. At 3-month follow-up, she remains in WHO functional class III with 6MWD of 310 metres and NT-proBNP 1,800 pg/mL. She has not improved to low-risk status. What escalation is recommended?

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Correct answer: D — Add parenteral prostacyclin therapy (e.g. intravenous epoprostenol or subcutaneous treprostinil)

The 2022 ESC/ERS PH Guidelines use a treat-to-target approach based on risk stratification. Patients who remain at intermediate or high risk after initial combination therapy (inadequate treatment response) should be escalated to triple therapy by adding a prostacyclin pathway agent. Parenteral prostacyclins (IV epoprostenol, SC treprostinil) are recommended for patients failing to achieve low-risk status on dual oral therapy (Class I). Adding sildenafil to ambrisentan + tadalafil would combine two PDE5 pathway agents and is not standard. Switching between ERAs (ambrisentan to bosentan) provides no escalation. Lung transplant referral should be considered alongside medical escalation, not instead of it.

Reference: ESC/ERS (2022): Guidelines for Pulmonary Hypertension