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Transplant Referral in PAH — EECC MCQ

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HardPulmonary Vascular DiseaseTransplant Referral in PAHEECC

A 60-year-old man with IPAH on triple combination therapy (ERA + PDE5i + IV epoprostenol) remains in WHO FC III-IV with a high-risk profile. Lung transplantation is discussed. What is the timing recommendation for transplant referral in PAH?

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Correct answer: B — Referral for transplant assessment should occur at the time of inadequate treatment response (intermediate-high risk despite maximal therapy), not as a last resort

The 2022 ESC/ERS PH Guidelines recommend early referral for transplant assessment (at the time of inadequate treatment response to maximal PAH-specific therapy, not as a last resort). This is critical because: (1) transplant waitlist times are long (months to years); (2) patients who deteriorate to WHO FC IV have very high mortality; (3) early referral allows assessment and listing while the patient is still in a condition to undergo transplantation. Options include bilateral lung transplant (most common for IPAH) or heart-lung transplant (when RV failure is severe and irreversible). The REVEAL and French registry risk scores help identify patients who should be referred. Bridge to transplant with IV prostacyclins or mechanical support (ECMO) may be needed.

Reference: ESC/ERS (2022): Guidelines for Pulmonary Hypertension; ISHLT Lung Transplant Guidelines