Pulmonary arterial hypertension (PAH) occurs when the arteries in the lungs have elevated blood pressure, and the right side of the heart has to work harder to pump blood to the lungs. Over time, as the heart must continue to work harder, the muscle weakens and heart failure occurs.
Though PAH isn’t curable, it’s treatable with medications and lifestyle changes.
There are two broad types of pulmonary arterial hypertension:
- idiopathic PAH
- secondary PAH
Both types are very rare.
With idiopathic PAH, there’s no known cause for the condition. It seems to have developed on its own, though sometimes there’s a genetically inherited component. In secondary PAH, the condition has developed due to some other underlying condition.
- shortness of breath
- dizziness or fainting
- racing pulse or heart palpitations
- chest pain
- abdominal swelling, or swelling of the arms, legs, or ankles
- blue or chalky skin, especially at the fingertips
Anyone can develop PAH. There are a number of risk factors that can make someone more susceptible to this and other heart and lung conditions, such as:
- family history
- other health conditions
- drug use
- high altitude
Though PAH can strike at any age, it’s almost exclusively found in adults. The most common window is between 20 and 60 years of age.
As for gender, idiopathic PAH is twice as common in women as in men, according to the Pulmonary Hypertension Association. Younger women of childbearing age are especially likely to develop the condition.
Idiopathic PAH develops without any visible cause. In some cases, family history seems to play a role, which would indicate a genetic link. In fact, a genetic marker has been found for PAH.
However, not everyone with idiopathic PAH has this marker, or any family history of the condition. Learn why it’s important to understand your family health history.
Other health conditions that are risk factors for PAH include:
- obesity and sleep apnea, which deprive the body of oxygen, forcing the heart to work harder to pump blood to the lungs for oxygenation
- HIV infection
- heart and lung conditions, including chronic obstructive pulmonary disease (COPD) and diseases of the left side of the heart
- history of blood clots in the pulmonary arteries
- liver disease
- lupus and other diseases of the connective tissues
- pregnancy, which seems to slightly raise the risk of PAH
Women already living with PAH who become pregnant have a high risk of death due to heart failure. Women with PAH should discuss the risks thoroughly with their doctor if considering becoming pregnant.
Recreational drugs such as methamphetamines and cocaine, as well as diet drugs such as Fen-Phen, have been shown to cause PAH. Sometimes the condition doesn’t develop until many years after drug use has ended.
Those who live at high altitude for extended periods are more likely to develop PAH. Research has shown that this condition can be reversed if the person moves back near sea level. Those who already have PAH and travel to high altitudes may find that their symptoms worsen during their trip.
Many of these risk factors are difficult to control, if not impossible. What you can definitely control is how you react to symptoms of PAH or other heart and lung conditions.
Visit your doctor at the first signs of trouble. There are several diagnostic tests that can pinpoint the cause of your symptoms, and a number of treatment options exist. Treatment of PAH generally involves managing symptoms of the condition, using medications and lifestyle changes.