Evolving profile and outcomes of patients with pulmonary arterial hypertension (PAH) undergoing lung transplantation in France, Journal of Heart and Lung Transplant, September 3, 2026

A study published in Journal of Heart and Lung Transplant Open by a large French team looked at all 241 patients with pulmonary arterial hypertension who had a lung or heart-lung transplant in France between 2008 and 2023. Using data from the national registries for the disease and for transplants, the authors compared two periods: 2008 to 2015 and 2016 to 2023.

Good news

  • Older and sicker patients can now be transplanted safely. The share of patients aged 50 or over rose from 30% to 44%, yet survival three months after transplant improved from 85% to 91%.
  • Shorter waits. The usual wait on the transplant list almost halved, from 194 to 103 days.
  • Fewer emergencies. The share of patients needing an urgent transplant fell from 55% to 42%, probably because doctors now assess risk better and refer patients at the right time. Even when a transplant is urgent, results are no worse.
  • Temporary heart-lung support (ECMO) is used more and more. This machine takes over the work of the heart and lungs for a while by pumping and oxygenating the blood outside the body. Its use as a “bridge” for critically ill patients waiting for new lungs more than doubled, from 6% to 14%, and the authors say it has profoundly changed how these patients are managed. By stabilising a failing right heart, it widens the window of time in which a transplant is still possible, and its increasing use did not affect survival after transplant. About a third of patients also need it just after surgery, to give the heart time to adapt to the new lungs.
  • Double lung transplant is now the norm. Heart-lung transplants fell from 21% to 5%, because the heart recovers well once the new lungs are in place.
  • Medicines are delaying the need for transplant. More patients were on triple therapy before transplant (56%, up from 42%). One patient was even taken off the list after improving on sotatercept, and new treatments may further reduce the need for transplants in future.

Challenges that remain

  • Survival at one year has not improved, at about 75%, and remains lower than after transplants for other lung diseases (80 to 88%). Age was the only factor linked to a higher risk of death in the first year.
  • Access is very unequal. For every patient transplanted, more than five patients aged 65 or under died without one. This varies widely by type of disease: about one transplant for every nine deaths in patients with connective tissue disease, compared with 1.5 transplants for every death in the inherited form. Patients with liver-related pulmonary hypertension were almost never transplanted.
  • Who misses out. Patients who died without a transplant were more often men, older, heavier, smokers, and had other illnesses, all factors that can rule out or delay a transplant. Women may also face longer waits, as smaller donor lungs are needed.
  • Late referral is a risk. Patients with idiopathic pulmonary arterial hypertension did worse than those with the inherited form or pulmonary veno-occlusive disease, probably because they are often treated with medicines for longer before being referred.

Key messages for patients

  • Risk scores alone don’t tell the whole story: a patient who is “low risk” only thanks to intensive treatment, such as intravenous prostacyclin and triple therapy, is still in a fragile balance and should be considered for referral to a transplant centre.
  • Care in expert centres makes a difference: advanced support such as temporary heart-lung support is only available in specialised centres, and in France about three quarters of these transplants are done in a single specialist hospital (Marie Lannelongue).

The authors note that the findings reflect France’s highly centralised system and may not apply directly to other countries.

Read more at this link on https://pubmed.ncbi.nlm.nih.gov/42780297/

Citation

Pradère P, Luong MC, Le Pavec J, Issard J, Coiffard B, Renard S, Boulate D, Renaud-Picard B, Olland A, Villeneuve T, Demant X, Maury JM, Chatron E, Mitilian D, Dauriat G, Boucly A, Sitbon O, Jaïs X, Montani D, Humbert M, Fadel E, Mercier O, Horeau-Langlard D, Savale L. Evolving profile and outcomes of patients with pulmonary arterial hypertension undergoing lung transplantation. JHLT Open. 2026 Sep 3;14:100675. doi: 10.1016/j.jhlto.2026.100675. PMID: 42780297; PMCID: PMC13597181.

TRANSLATE »
Scroll to Top