What is PAH?

Pulmonary arterial hypertension (PAH, WHO Group 1) is a serious condition that creates high blood pressure in the arteries of your lungs. It makes it difficult for blood to flow through your lungs, which can force your heart to work harder than normal. PAH has no cure and gets worse over time.

How does PAH happen?

There are several causes of PAH that have been identified, including connective tissue disease (CTD), congenital heart defects (CHD), liver disease, and other health conditions. When the cause is unknown, as is most common, the disease is referred to as idiopathic PAH.

It is also possible for gene mutations that cause PAH to be passed down, which is known as heritable PAH. Certain medicines and narcotics have also been found to cause the disease.

How is PAH diagnosed?

Right heart catheterization is needed for a conclusive diagnosis. PAH symptoms often resemble those of other heart and lung conditions, so doctors typically eliminate the possibility of these other diseases when evaluating for PAH.

What are the symptoms of PAH?

The strain PAH puts on your heart and lungs can cause symptoms like chest pain, shortness of breath, feeling tired, swollen ankles or legs, and dizziness.

How common is PAH?

PAH is a rare disease, but researchers differ on how to properly determine the true incidence of PAH. However, some estimates suggest that between 500 and 1000 new cases of idiopathic PAH are diagnosed each year in the US.

Can COVID cause PAH?

While it has been suggested that PAH could be a consequence of COVID-19, there is currently no data to confirm a causal relationship. Not all causes of PAH are known.

Who is at risk of PAH?

PAH can affect anyone, but it is most common in women, non-Hispanic Black people, and people over the age of 75.

How is PAH treated?

Small blood vessels in your lungs produce 3 substances that make up the foundational pathways: nitric oxide, endothelin, and prostacyclin. The right balance of these subtances enables red blood cells to flow freely through the lungs and carry oxygen to the rest of the body. Treatments for PAH may target one or more of these or other pathways in order to restore balance.

When you have PAH, 1 or more of these substances becomes out of balance. Currently, there is no test available to determine which substance needs to be adjusted. Some patients may benefit from treatment addressing more than 1 pathway.

What are the three foundational pathways?

PAH can cause imbalances in three naturally occurring substances in your body - nitric oxide, endothelin, and prostacyclin.

Is there a cure for PAH?

PAH can be managed with medication, but there is currently no cure.

What types of doctors treat PAH?

Most doctors who treat patients with PAH are cardiologists or pulmonologists. Your care team may also include a primary care physician, respiratory therapist, and other healthcare practitioners.

Can PAH be reversed?

PAH is a progressive disease, which means that it gets worse over time. However, when effective medical interventions are taken, the symptoms of PAH can become more manageable.