One to 3% of about 5,000 lung transplants each year around the world are for pulmonary hypertension, according to Nicholas Kolaitis, a transplant pulmonologist at University of California-San Francisco. That’s about 50 to 150 lung transplants worldwide for PH each year.

Historically, new lungs were matched with recipients in the U.S. based on a person’s time on the lung transplant wait list. But in 2005, in an effort to create a fairer system, the United Network of Organ Sharing changed the way lungs are distributed.

Now the “urgency” system gives a score to each person based on the severity of their disease and likely success of transplant surgery. The score is based on several medical tests.

While the newer system is an improvement, there are still some inequities for people with PH, Kolaitis says. That’s because the measurements that define “urgency” don’t favor those with PH. For example, people with PH aren’t affected much by measurements such as lung size or carbon dioxide level. Those measurements are more relevant in diseases of the lung tissue, such as chronic obstructive pulmonary disease, not diseases of the lung vessels like PH.

After the 2005 changes, every lung disease reflected on the transplant list — except PH — saw fewer people dying while still on the list. While UNOS has made revisions since then to try to address this problem, people with PH are still getting lung transplants later in their disease, when they are very sick, Kolaitis says.

“The fact that patients with pulmonary hypertension need to be sick enough to overcome the disadvantage of the organ allocation means that we’re transplanting patients with pulmonary hypertension later than we should be, which leads to poor outcomes after transplantation,” he says. “So this needs to be revised from an advocacy standpoint.”

Lung or heart-lung transplant

Lung or heart-lung transplantation provide an option for some individuals who are no longer responding significantly to available medical therapies for pulmonary hypertension. Transplantation may extend and improve a patient’s quality of life but requires life-long care and medications.

Transplants