Is pulmonary arterial hypertension reversible?

Is it reversible?

While there is no cure for PAH, treatment options may improve the quality of life and outcomes in people with the condition.

Developments in medical therapies have also shown evidence of potentially reversing PAH.

For example, a 2018 study investigating the effects of a drug on the HIF-2α gene found that the drug effectively reversed right heart failure and vascular remodeling in rodents with PAH. The HIF-2α gene promotes arterial wall thickening, which is a key step in the development of PAH. However, the drug does not cure PAH.

Vascular remodeling is a change to the structure of the cells and fibroblasts in the arteries.

However, PAH is often idiopathic, meaning it occurs spontaneously, without any underlying or known cause. Idiopathic PAH or PAH associated with underlying lung or connective tissue disease is more difficult to reverse or treat.

Treatment

Treatment options for PAH aim to improve symptoms, increase exercise capacity and slow disease progression. The suitability of a treatment option will depend on a number of factors, including the severity of PAH.

Medication

A number of drugs exist to treat PAH. These include:

• calcium channel blockers, such as amlodipine or nifedipine
• endothelin receptor antagonists, such as ambrisentan
• phosphodiesterase type 5 inhibitors, such as sildenafil and tadalafil
• prostacyclin analogs, such as epoprostenol

Oxygen therapy

Oxygen therapy involves administering oxygen to a person with low blood oxygen levels.

A 2021 study found that supplemental oxygen may have therapeutic benefits for people with PAH. This is because oxygen is a pulmonary vasodilator, meaning it expands the vessels of the lungs.

Lung transplantation

Lung transplantation involves the replacement of a diseased lung with a healthy lung from a donor. Transplantation may involve the replacement of a single lung or both lungs.

A small 2018 study found that lung transplantation provides good long-term and short-term survival for individuals with PAH.

Surgery

Some surgical options may provide better symptom control and exercise capability in people with PAH.

For example, atrial septostomy is a surgical procedure that involves creating a hole between the upper chambers of the heart. This allows the blood to flow from the right side of the heart to the left side, reducing pressure on the right side.

A small 2023 retrospective study involving 12 people with PAH and severe right heart failure found that this surgical option improved survival rates.

Pulmonary rehabilitation

Pulmonary rehabilitation is a program that typically involves breathing techniques, exercises, and education to help improve the physical and psychological health of individuals with respiratory conditions and diseases.

Pulmonary rehabilitation aims to improve physical function, reduce symptom impact, and improve quality of life.

A 2020 study found that treatment programs that incorporate exercise significantly improve exercise capacity and quality of life in individuals with PAH.

PAH can cause death if left untreated.

The mean survival rate for untreated idiopathic PAH is 2-3 years from the PAH diagnosis.

Summary

PAH is a condition that affects the pressure in the blood vessels between the heart and the lungs.

It might be possible to reverse PAH that has a known cause by treating the underlying condition.

Treatment options for PAH include medication, surgery, and oxygen therapy.