Pulmonary Arterial Hypertension (PAH)

• Connective tissue disorders like lupus and scleroderma

• Congenital heart defects/abnormalities

• Exposure to certain toxins or drugs (including cocaine and methamphetamines)

• Infections, including HIV and schistosomiasis (a disease caused by parasitic flatworms)

• Cirrhosis of the liver

When the cause is unknown, it is called idiopathic pulmonary arterial hypertension.

You are at a higher risk of getting pulmonary arterial hypertension if:

• You are a young female of childbearing age (idiopathic PAH is seen more often in this demographic than any other)

• You have a family history of pulmonary arterial hypertension

• You have scleroderma, a connective tissue disorder, cirrhosis of the liver, or HIV

• You use cocaine or methamphetamines

Symptoms of pulmonary arterial hypertension can often mimic other respiratory ailments and diseases like emphysema, COPD, and asthma, or heart failure. PAH symptoms include:

• Shortness of breath

• Fatigue

• Chest pain/pressure

• Racing pulse

• Dizziness

• Swelling in the ankles, legs, and abdomen

• Bluish color in lips and skin

It’s important to note, PAH can also have the same/similar symptoms as other respiratory diseases. If you’re concerned about your symptoms, call your doctor for a full health screening.

If you are having one or more of the symptoms noted above, see your doctor. They will ask you for a full medical history including questions pertaining to elevated risk factors and your specific symptoms. Depending on whether PAH is suspected, your doctor may order one or more of the following tests:

• Echocardiogram (an ultrasound of the heart that checks blood pressure in the pulmonary arteries)

• CT scan

• Ventilation-perfusion scan (a test that helps find blood clots)

• Chest x-ray

• Electrocardiogram (EKG or ECG; this test traces the heart’s activity and shows whether the right side of the heart is strained)

• Stress or exercise test

If tests show that you’re at risk for pulmonary arterial hypertension, your doctor may perform a right heart catheterization to be sure. This is when the physician places a catheter into a large vein (usually the jugular in the neck or the femoral in the leg) and threads it into the right side of your heart. A monitor then records the right-side heart pressure and the pressure in the pulmonary arteries.

Pulmonary arterial hypertension has no cure, but with proper treatment, symptoms can be managed to encourage a full and healthy lifestyle. If diagnosed with PAH, your physician will work closely with you to develop a treatment plan that’s unique to your needs. The first step to treating PAH is to find the cause. For example, if the cause of your pulmonary arterial hypertension is lupus, your doctor will first treat that to help manage and improve your PAH.

The most common treatment for pulmonary arterial hypertension is oxygen therapy. During oxygen therapy, you breathe pure oxygen through fitted prongs inserted into your nose. This will help if you become short of breath with low blood oxygen levels.

If your PAH is advanced or severe, your physician may prescribe calcium channel blockers, medicines that lower blood pressure in the lungs and other parts of the body. If those do not help, your physician may refer you to a specialized treatment center or prescribe other medications/treatments including:

• Oral medications

• Inhalers

• IV drugs

In very severe cases, your doctor may consider a lung transplant.

If you have pulmonary arterial hypertension, one of the best ways to manage this condition is to take good care of yourself. You can do this by:

• Getting regular exercise to increase pulmonary health and strengthen your heart/lungs

• Eat a healthy diet

• Get proper rest

• Quit smoking (if you smoke)