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PAH Therapy De-escalation Risks — EECC MCQ

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HardPulmonary Vascular DiseasePAH Therapy De-escalation RisksEECC

A 55-year-old woman with idiopathic PAH on ERA + PDE5i + SC treprostinil has stabilised in WHO FC II with good haemodynamics (mPAP 32, PVR 3.5 WU, CI 3.2). She asks about discontinuing treprostinil (which requires continuous SC infusion and causes painful site reactions). Can PAH therapy be de-escalated?

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Correct answer: E — De-escalation of PAH therapy is EXTREMELY high-risk — abrupt withdrawal of prostacyclin can cause fatal rebound PH crisis; any de-escalation should only occur at expert centres with very gradual dose reduction, close monitoring, and immediate re-escalation capability if clinical deterioration occurs

PAH therapy de-escalation is one of the most controversial areas in PH management. The 2022 ESC/ERS PH Guidelines: (1) PAH is a CHRONIC disease requiring LIFELONG therapy — achieving low-risk status does NOT equate to cure; (2) abrupt withdrawal of prostacyclin therapy (IV epoprostenol, SC treprostinil) can trigger fatal rebound pulmonary hypertensive crisis within hours-days (rebound phenomenon from upregulation of vasoconstrictive pathways during treatment); (3) GRADUAL dose reduction may be cautiously attempted in HIGHLY selected patients at EXPERT centres only — with: very close haemodynamic monitoring, immediate re-escalation capability, and shared decision-making; (4) the evidence base for safe de-escalation is minimal — mostly case series. Most PAH experts recommend maintaining triple therapy once achieved, particularly prostacyclin, and focusing on symptom management (site reaction management, device optimisation) rather than drug withdrawal.

Reference: ESC/ERS (2022): PH Guidelines