Successful lung transplants can dramatically improve quality of life for people with pulmonary hypertension and extend their lives by several years. Not everyone with PH will need one, but for some people, a double-lung transplant may be the next life-giving step.
“Lung transplant becomes an option for people with PAH or other advanced pulmonary diseases when they have failed maximal medical management and their disease is progressing,” says Jamie L. Todd, a transplant pulmonologist at Duke Health in North Carolina.
Progression could mean poorer right heart catheterization measurements and decreasing six-minute walk distance. In addition, the person could have worsened symptoms and a declining quality of life, Todd says.
For people with PH, lung transplant generally means a double-lung transplant because the disease affects both lungs. The average survival rate after double-lung transplant is about seven years, according to the National, Heart, Lung and Blood Institute. The first year is the most critical.
Data from 2020 show that 90% of lung transplant recipients will be alive one year later, while 61% will be alive at five years post-transplant, and 33% will be alive at 10 years.*
Getting a heart and lung transplant for pulmonary arterial hypertension is less common because studies have shown that a lung transplant by itself often is sufficient. New lungs allow the previously struggling heart to improve.
Beyond surviving, lung transplant recipients often thrive. Quality of life can improve dramatically with higher energy levels, better mental health and the ability to do everyday activities, exercise and travel. After transplant, people no longer are tethered to pumps and catheters or supplemental oxygen.
Transplant candidates
The International Society for Heart and Lung Transplantation recommends people on IV or subcutaneous prostacyclin therapy or those with worsening PAH be referred to a transplant team.
The earlier a person with PH is referred to a transplant center to learn about the process, the better, transplant pulmonologists say. When referred early, patients have more time to consider what a transplant means, ask questions, get medical evaluations and address barriers to transplant — even if they never need the surgery.
“It’s better to have all the information earlier rather than when you’re in a situation of medical duress,” says Matthew R. Pipeling, a transplant pulmonologist at Duke Health in North Carolina.
Ideally, lung transplants should take place before other organs are damaged. For example, PAH can cause kidney and liver disease.
Medical teams at transplant centers should decide whether someone is a good candidate for transplant, these transplant pulmonologists say. If a PH doctor says a patient isn’t a candidate for transplant, the patient can ask for a referral to a transplant center, even if their PH is relatively stable.
“Being referred for transplant does not mean that you are getting a transplant and does not mean that you need a transplant right now,” says Nicholas Kolaitis, a transplant pulmonologist at University of California-San Francisco. “Being referred for transplant and meeting with the transplant team should be viewed as an educational opportunity to learn about transplant and whether it’s something you might want in the future if you actually need it … The worst thing that happens is a patient is referred to transplant too late.”
Transplant pulmonologists and centers base transplant candidacy on several medical, surgical and social factors. A medical evaluation looks at other health conditions, kidney or liver damage, or heart disease. A surgical evaluation examines whether a person is fit enough to survive surgery.
Candidates must have a social support system to help them recover from surgery and perform everyday tasks such as dressing, organizing medications, grocery shopping and traveling to pulmonary rehabilitation and doctor appointments.
Rejection and infection
Risks after lung transplant surgery fall into two areas: rejection and infection.
Todd tells her patients that for the first six months after transplant their full-time job is to get better. That means going to all pulmonary rehab appointments, improving nutrition, learning the new medication regimen and working with the transplant pulmonology team to deal with side effects.
Transplant is not perfect. The way that I view transplant is it’s trading one disease for another.
There are three primary types of organ rejection. About 30% of lung transplant recipients will experience “acute rejection” in the first year after transplant, Kolaitis says. In those cases, most people get better after taking high doses of steroids.
“Antibody-mediated rejection” is rare and is treated with chemotherapy-type drugs. Chronic rejection” can happen months or years after transplant. There are medications to treat chronic rejection, but they don’t work in everyone. If lungs begin to fail and don’t respond to treatment, it’s rare for someone to go back on the list for a second transplant.
However, the biggest risk after transplant — and the most common cause of death within the first year — is infection. Transplant recipients must take medications to suppress their immune system for the rest of their lives. After transplant, the immune system sees the new lungs as an invading virus to attack, which could lead to the body rejecting the lungs. Medications that suppress the immune system reduce that risk.
Because of those medications, transplant recipients will always be immunosuppressed. If they get sick, they are at higher risk of getting very sick. So it’s critical to try to avoid catching illnesses, especially respiratory viruses like COVID-19, flu and RSV.
Recommendations on how to avoid illness vary by transplant center. Plus, each person will need to make their own decisions on how they want to live their life and the risks they are willing to take, Pipeling says.
Transplant pulmonologists might recommend wearing a mask in large gatherings or avoiding crowds as much as possible. Family and friends should stay away when they are sick.
Some transplant centers recommend no alcohol, while others recommend one drink only a couple times a year. Alcohol can interact poorly with rejection medications, and even non-alcoholic beer has trace amounts of alcohol.
Transplant recipients shouldn’t eat uncooked fish (raw oysters or sushi), raw or undercooked eggs, or unpasteurized milk and cheese because of the risk of food poisoning. For the same reason, they must be careful eating at potlucks or buffets, where the food may have been out too long and/or not washed properly. Also, many times gardeners are asked to give up their hobby because of the disease risk from fungus in soil.
Lung transplant recipients will see their transplant pulmonologist for the rest of their lives, more often in the first year. “Transplant is a major commitment and a life-altering event,” Todd says.
But the impact on one’s health and quality of life can be phenomenal. “I’ve certainly seen people go back to work or volunteer in a role that’s meaningful to them,” she says. “We’ve had people go back to college and get degrees, travel, including international travel, again. I don’t think there’s much that people have said they want to do that they can’t do after transplant.”
*Valapour M, Lehr CJ, Skeans MA, et al. OPTN/SRTR 2020 Annual Data Report: Lung. Am J Transplant. 2022; 22(suppl 2):438-518.
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