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Bilateral Lung Transplant for PAH — EECC MCQ

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ModeratePulmonary Vascular DiseaseBilateral Lung Transplant for PAHEECC

A 55-year-old woman with PAH on triple combination therapy (ERA + PDE5i + SC treprostinil) remains in WHO FC III-IV. She is assessed for lung transplantation. What type of transplant is performed for PAH?

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Correct answer: D — Bilateral sequential lung transplantation is the standard procedure for PAH — the transplanted lungs accept the entire cardiac output, reversing pulmonary hypertension; RV function typically recovers rapidly post-transplant as afterload normalises

Lung transplantation for PAH per the 2022 ESC/ERS PH Guidelines and ISHLT guidelines: (1) bilateral sequential lung transplant is the procedure of choice (NOT single lung — single lung transplant causes ventilation-perfusion mismatch as blood preferentially flows to the transplanted lung); (2) RV recovery: remarkably, even severely dilated/dysfunctional RV typically recovers rapidly (days-weeks) once afterload is normalised by the transplanted lungs — therefore heart-lung transplant is rarely needed unless RV dysfunction is truly irreversible or there is concomitant uncorrectable cardiac disease; (3) timing: refer early (when inadequate response to maximal PAH therapy or high/intermediate-high risk profile), as waitlist times are months-years; (4) 5-year survival post-lung transplant for PAH: ~50-60% (worse than for some other lung diseases); (5) bridge strategies: IV prostacyclins, ECMO (increasingly used as bridge to transplant).

Reference: ESC/ERS (2022): PH Guidelines; ISHLT Lung Transplant Guidelines