Do You Have PAH? Here’s How to Tell

high blood pressure
If you have high blood pressure that manifests in the arteries of the lungs, this condition is called pulmonary arterial hypertension, or PAH. It is one form of the more broad condition of pulmonary hypertension, and one that you should take seriously if you have this condition.

The Role of Pulmonary Arteries

The arteries in your cardiovascular system carry blood from the heart to the lungs, where it gets the oxygen it needs to deliver to the rest of your body. If those arteries are abnormally constricted, your heart has to work significantly harder and beat faster, leading to higher blood pressure within the lungs.

Why it Matters

This condition could occur on its own, or at the same time as a wide variety of other medical conditions, and even could result from certain medications. Left untreated, the condition often worsens over time and brings blood pressure in the pulmonary arteries to a dangerously high level, straining the heart and potentially becoming life threatening. While there is no cure, there are medications available to treat the symptoms so it’s critical that you find out if you have this disease and seek treatment.

Symptoms of PAH

The most common symptoms a patient experiences are chest pain, dizziness, fainting, fatigue, swelling in the legs, shortness of breath or lightheadedness during physical activity, and overall weakness. A doctor will generally diagnose this disease with chest X-rays, electrocardiogram (EKG), echocardiogram, walking tests, lung scans, and blood tests.

Causes

For some the cause of PAH is unknown, while others likely develop it as a hereditary disease linked to a genetic defect. More commonly, the disease is associated with one or more other conditions, such as:

  • Autoimmune diseases (i.e., lupus)
  • Congenital heart or lung disease
  • Liver disease
  • HIV
  • Illegal drugs or OTC supplements (i.e., appetite suppressants, cocaine)
  • Thyroid disorders and metabolic conditions
  • Gaucher disease, and other medical conditions

Women are more likely to get this disease than men, and it can worsen during labor and delivery and result in maternal health risks.

Treatment Options

There is no cure for this disease, and it can worsen over time, so getting treatment as soon as possible is essential. The most common treatments are medication and surgery, including the use of endothelin receptor antagonists (ERAs) that stop harmful effects of endothelin, like Letairis (generic name ambrisentan). These treatments can reduce the symptoms you experience, improving quality of life and slowing the progression of the disease.

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