Primary pulmonary hypertension, also called pulmonary arterial hypertension (PAH), occurs when arteries in the lungs become thick and narrow. This raises blood pressure in the lungs by restricting blood flow.
The condition also causes a person’s heart to work harder to pump blood through the narrowed arteries. Eventually, a person’s heart may lose the ability to effectively pump blood throughout the body.
PAH is a type of pulmonary hypertension, or high blood pressure in the lungs. Additionally, it is a rare, progressive disorder. This means it worsens over time. Treatments typically focus on managing symptoms and improving quality of life.
The condition most commonly affects females between the ages of 30 and 60, reports the American Lung Association. Healthcare professionals diagnose about 500–1,000 new cases of PAH in the United States each year, notes the association.
This article reviews the symptoms, causes, diagnosis, and treatment of PAH. It also discusses the potential outlook for someone with PAH.
A note about sex and gender
Sex and gender exist on spectrums. This article will use the terms “male,” “female,” or both to refer to sex assigned at birth. Click here to learn more.
PAH can cause several symptoms. They may appear at any stage in a person’s life and can worsen over time.
At first, a person may not notice any symptoms, or they may believe the symptoms have another cause, such as other lung conditions.
As the disease progresses, a person may develop signs and symptoms that include:
- chest pain
- increased shortness of breath
- swelling of the legs and feet, and eventually the neck and abdomen
- lips and fingers turning blue
- dizziness and fainting spells
- heart palpitations
The symptoms of PAH are nonspecific. This means several different diseases may cause the symptoms to appear. As a result, a doctor may misdiagnose the condition as other common lung diseases, such as chronic obstructive pulmonary disease (COPD) or asthma.
However, PAH is a rare condition. A person is more likely to have a condition like asthma or COPD than PAH.
Healthcare professionals do not know what exactly causes PAH. Other forms of pulmonary hypertension
The condition may occur over time, following injury to the cells that line the blood vessels of the lung. If the cause of this injury is unknown, doctors refer to the condition as idiopathic PAH.
If healthcare professionals believe a genetic mutation inherited from a parent has led to the injury, doctors refer to the condition as heritable PAH. According to the American Lung Association, approximately 15–20% of people with PAH have heritable PAH.
Some other conditions associated with the development of PAH include:
- connective tissue disorders, such as lupus and scleroderma
- exposure to certain toxins and drugs, including cocaine and methamphetamine
- congenital heart abnormalities
- infections, including HIV and schistosomiasis
Since there are similarities in symptoms between PAH and other common lung conditions, diagnosis can be challenging for healthcare professionals.
The diagnostic process typically involves eliminating other possible causes of a person’s symptoms.
A healthcare professional will likely order several tests, which may include:
- blood tests to check for a range of underlying conditions
- electrocardiogram to check for abnormal electrical signals in the heart
- lung function test to check for COPD, asthma, or similar conditions
- 6-minute walk test to check how oxygen levels respond when someone is physically active
- echocardiogram, in which a healthcare professional uses ultrasound to check the chambers of the heart and
estimatethe blood pressure level in the pulmonary arteries
- right-heart catheterization, which is an invasive test that measures the blood pressure in the lungs
A doctor may also order genetic testing to check for genetic mutations associated with PAH. The presence of the condition in one or more family members can help a doctor confirm the diagnosis.
Delays in PAH diagnosis may occur for several reasons, including:
- symptoms often start mild and nonspecific
- younger people and people with a history of other lung disorders often have delayed diagnosis
- as the condition progresses, a doctor may mistake it for other conditions, such as congestive heart failure
A person who has not received a PAH diagnosis should talk with a doctor if they experience:
- symptoms that do not improve with traditional treatments for COPD, asthma, or other common conditions
- chest pain or fainting with exertion
- extreme fatigue
- unexplained swelling in the abdomen and legs
There is no cure for PAH. Treatments focus on improving quality of life and slowing the progression of the disease.
Early diagnosis may help improve a person’s outcome. Seeing a specialist at a pulmonary hypertension care center may help with getting a diagnosis and tailoring a treatment plan.
Some common treatment options include:
- endothelin receptor antagonists, which help prevent blood vessels from narrowing
- phosphodiesterase inhibitors (PDE5 inhibitors), which allow the lungs to relax and blood vessels to widen
- prostacyclin analogue, selective IP receptor agonist, and soluble guanylate cyclase (sGC) stimulators, which help the blood vessels in the lungs relax
- diuretics, which help treat swelling and fluid retention
- anticoagulants, which help prevent blood clots
- portable oxygen tank, which helps maintain oxygen levels
- lung or heart transplant, in severe cases
A person can also take steps to manage PAH at home, including:
- staying active and exercising regularly
- eating a nutrient-dense diet
- avoiding or quitting smoking, if applicable
- avoiding hot tubs, saunas, and traveling to high altitudes
- discussing pregnancy with a doctor before becoming pregnant
- seeking support groups and help for mental health conditions that may develop
People should speak with a doctor if they notice their symptoms worsening or changing. A doctor may be able to help a person find new therapies that work for them and suggest additional lifestyle strategies.
PAH does not have a cure. Medications, therapies, and lifestyle adjustments can help a person manage their condition long term.
Early diagnosis may help improve a person’s survival. People may want to consider working with a specialist to help receive a prompt diagnosis and appropriate treatment.
A person may improve their outlook by following their treatment plan and taking steps to manage the condition at home.
PAH is a rare type of pulmonary hypertension. It may cause several, nonspecific symptoms at first. Doctors do not know exactly what causes the condition, but genetics may play a role in some cases.
Diagnosis may be challenging as the symptoms of PAH are similar to other lung conditions. Early treatment may improve a person’s quality of life as well as slow disease progression.
A person should consider speaking with a healthcare professional if they experience any of the symptoms of PAH.