Symptoms, Diagnosis and Treatment

What are the Symptoms of PPH?

PPH can develop slowly. In fact, symptoms can be very minor for years. Symptoms may include:

  • fatigue
  • racing heartbeat
  • chest pain

Symptoms become more severe and limiting as the disease worsens. Those symptoms may include:

  • feeling lightheaded or short of breath after routine activity like climbing two flights of stairs
  • fainting
  • swelling in your legs and ankles
  • bluish lips and skin

How is PPH Diagnosed?

It is difficult for doctors to detect PPH in a routine medical examination. Even when the disease has progressed, the signs and symptoms may very similar to other conditions that affect the heart and the lungs.

To determine if you have pulmonary hypertension, your doctor may recommend one or several tests that help rule out other disorders that could be causing your symptoms.  Your doctor will diagnose PPH based on:

  • your medical and family history
  • a physical exam
  • results from tests and procedures -- the tests and procedures also will help your doctor determine the cause of your PPH, and its severity.

Tests may include:

  • Echocardiography (an ultrasound picture of the heart to help determine how well it is working)
  • Chest x ray (of your heart and lungs)
  • EKG (electrocardiogram; an electrical measure of your heart rate and how regularly your heart is beating)
  • Right heart catheterization (use of a special device that the doctor threads through a vein into the heart and arteries of the lungs to measure pressure in the arteries and test how well the heart is pumping)
  • Pulmonary function tests (breathing tests that measure lung function)
  • Overnight oximetry (measures the level of oxygen in your blood overnight)
  • Lung ventilation/perfusion scan (measures air and blood flow in your lungs)
  • Blood tests rule out other diseases, such as HIV, liver disease, and autoimmune diseases like rheumatoid arthritis.
  • Exercise testing (a measure of a patient's ability to exercise)

How is PPH Treated?

PPH has no cure, but treatments may help relieve symptoms and slow the progress of the disease. Treatment will depend on how severe your PPH is. The earlier PPH is treated, the easier it is to control.  Treatment may include medication, surgical procedures, and lifestyle changes.


Primary pulmonary hypertension is treated with a number of drugs. None of the drugs cure PPH, but they may relieve symptoms. Your doctor will monitor you closely to see how you respond to a particular drug. Medications may include:

  • Diuretics, or "water pills." These medicines help reduce fluid buildup in your body, including swelling in your ankles and feet.
  • Blood-thinning medicines. These medicines help prevent blood clots from forming or getting larger.
  • Digoxin. This medicine helps the heart beat stronger and pump more blood.

Your doctor also may recommend oxygen therapy. This treatment raises the level of oxygen in your blood.

Surgical Procedures

  • Transplantation—Patients with severe PPH may be candidates for a lung transplant or heart-lung transplant.
  • A trial septostomy—In this treatment, a surgeon creates a hole between the top chambers of the heart. This allows blood to be pumped to the rest of the body without going through the lungs first. This can reduce strain on the heart, but it also greatly reduces the amount of oxygen that can be delivered to the body.

Lifestyle Changes

Make lifestyle changes, such as eating healthy, quitting smoking, and doing regular physical activity.

  • Nutrition—Fuel your body with a nutritious diet that is low in fat, cholesterol and sodium and rich in high-fiber, whole grain, foods—including fruits and vegetables—and lean protein.
  • Physical activity—Regular activity may help improve your ability to be active. Your doctor can help you create an exercise plan that's safe for you.
  • Smoke-free living—If you smoke, quit. Avoid secondhand smoke. Smoking and breathing secondhand smoke make PPH symptoms worse. Ask your doctor about products that can help you quit smoking. Get free, expert advice and support from the American Lung Association's Freedom From Smoking® program—available online.

Learn more tips for Living With PPH.