Criteria for lung transplantation and listing in patients with pulmonary arterial hypertension
| Referral |
| Potentially eligible patients for whom LTx might be an option in case of treatment failure |
| ESC/ERS intermediate–high or high risk or REVEAL risk score >7 on appropriate PAH medication |
| Progressive disease or recent hospitalization for worsening PAH |
| Need for i.v. or s.c. prostacyclin therapy |
| Known or suspected high-risk variants, such as PVOD or PCH, systemic sclerosis, or large and progressive pulmonary artery aneurysms |
| Signs of secondary liver or kidney dysfunction due to PAH or other potentially life-threatening complications, such as recurrent haemoptysis |
| Listing |
| Patient has been fully evaluated and prepared for transplantation |
| ESC/ERS high risk or REVEAL risk score >10 on appropriate PAH medication, usually including i.v. or s.c. prostacyclin analogues |
| Progressive hypoxaemia, especially in patients with PVOD or PCH |
| Progressive, but not end-stage liver or kidney dysfunction due to PAH, or life-threatening haemoptysis |
ERS, European Respiratory Society; ESC, European Society of Cardiology; i.v., intravenous; LTx, lung transplantation; PAH, pulmonary arterial hypertension; PCH, pulmonary capillary haemangiomatosis; PVOD, pulmonary veno-occlusive disease; s.c., subcutaneous.