There is no cure for pulmonary arterial hypertension (PAH). However, treatment can lead to reduced symptoms and improved quality of life.
PAH occurs when the pressure within the blood vessels linking the heart and the lungs is high. This high pressure can cause the narrowing of tiny arteries in the lungs, restricting blood flow and damaging the right-hand side of the heart.
Symptoms of PAH may include:
- shortness of breath
- blue lips
- chest pain
- swelling in the ankles, legs, and abdomen area
PAH increases the risk of right heart failure and death. It is extremely rare. According to the American Lung Association, approximately 500-1,000 people receive a diagnosis of PAH each year in the United States.
Continue reading to learn whether PAH is reversible, what treatment options exist, and the outlook for a person with PAH.
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
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
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.
A number of drugs exist to treat PAH. These
- 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 involves
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.
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.
Pulmonary rehabilitation is a program that typically
Pulmonary rehabilitation aims to improve physical function, reduce symptom impact, and improve quality of life.
PAH can cause
The mean survival rate for untreated idiopathic PAH is
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.