skip to main content

PAH Triple Therapy Escalation — EECC MCQ

Instant feedback + full explanation. One question, done properly.

ModeratePulmonary Vascular DiseasePAH Triple Therapy EscalationEECC

A 50-year-old woman with PAH on dual combination therapy (ERA + PDE5i) is assessed at 6 months. She remains in WHO FC III with 6MWD 340 m (decreased from 380 m at baseline), rising NT-proBNP (800 → 1,400 pg/mL), and worsening haemodynamics (CI 2.2 → 1.8 L/min/m²). What is the next treatment step?

Educational content. Not a substitute for clinical judgement or local policy.

Reveal the answer and explanation

Correct answer: A — Escalate to triple therapy by adding a prostacyclin pathway agent — either IV/SC prostacyclin (epoprostenol, treprostinil) for high-risk patients, or selexipag (oral IP receptor agonist) for intermediate-risk patients; failure of dual therapy to achieve low-risk status mandates treatment escalation

The 2022 ESC/ERS PH Guidelines use a treat-to-target strategy: reassess at 3-6 months after treatment initiation; if not meeting comprehensive low-risk criteria, ESCALATE treatment. For this patient: worsening FC, declining 6MWD, rising NT-proBNP, and deteriorating haemodynamics indicate inadequate response to dual therapy → escalation to triple therapy. Options for the prostacyclin pathway agent: (1) HIGH-risk features (WHO FC IV, CI <2.0, RA pressure >14 mmHg): IV epoprostenol or SC/IV treprostinil (most potent); (2) INTERMEDIATE-risk: selexipag (oral IP receptor agonist — GRIPHON trial showed reduced morbidity) or inhaled treprostinil/iloprost. The ultimate goal is achieving and maintaining a comprehensive low-risk profile (WHO FC I-II, 6MWD >440 m, NT-proBNP <300, CI >2.5). If triple therapy fails: transplant assessment.

Reference: ESC/ERS (2022): PH Guidelines; GRIPHON Trial