Conditions & Treatments

Primary Pulmonary Hypertension

Pulmonary hypertension is a lung disorder in which the blood pressure in the pulmonary artery rises far above normal levels.

Primary Pulmonary Hypertension

What is primary pulmonary hypertension (PPH)?

Pulmonary hypertension is a lung disorder in which the blood pressure in the pulmonary artery rises far above normal levels.

Primary pulmonary hypertension has been associated with the appetite suppressants fenfluramine and dexfenfluramine (fen/phen), which were taken off the market in 1997. In the U.S., there are an estimated 500 to 1,000 new cases diagnosed each year, but the actual number of cases is unknown. It is most common in women between the ages of 21 and 40; however, anyone can develop it.

What are the causes of PPH?

The exact cause of PPH is unknown. Research has linked primary pulmonary hypertension to genetic or familial predisposition. Researchers believe the blood vessels are particularly sensitive to certain internal or external factors, and constrict, or narrow, when exposed to these factors, such as an immune system factor, or sensitivity to drugs or other chemicals.

Secondary pulmonary hypertension occurs as a result of the effects of other conditions which may include diseases of the heart or lungs, a blood clot in the lungs, or a condition called scleroderma.

What are the symptoms of PPH?

The following are the most common symptoms for pulmonary hypertension. However, each individual may experience symptoms differently. Symptoms may include:

  • Fatigue

  • Difficulty in breathing (dyspnea)

  • Dizziness

  • Fainting spells (syncope)

  • Swelling in the ankles or legs (edema)

  • Bluish lips and skin (cyanosis)

  • Chest pain (angina)

  • Racing pulse

  • Trouble getting enough air

  • Palpitations, strong throbbing sensations brought on by increased heart rate

More severe symptoms indicate a more advanced disease. In advanced stages, the patient:

  • Is able to perform minimal activities

  • Has symptoms even when resting

  • May become bedridden if the disease becomes worse

The symptoms of primary pulmonary hypertension may resemble other conditions or medical problems. Consult your doctor for a diagnosis.

How is PPH diagnosed?

Pulmonary hypertension is rarely discovered in a routine medical examination, and in its later stages, the signs of the disease can be confused with other conditions affecting the heart and lungs.

Pulmonary hypertension is a diagnosis of exclusion. Diagnostic procedures may include:

  • Electrocardiogram (EKG or ECG). A test that records the electrical activity of the heart, shows abnormal rhythms (arrhythmias or dysrhythmias), and detects heart muscle damage.

  • Echocardiogram (echo). A procedure that evaluates the structure and function of the heart by using sound waves recorded on an electronic sensor that produce a moving picture of the heart and heart valves.

  • Pulmonary function tests. Diagnostic tests that help to measure the lungs' ability to move air into and out of the lungs effectively. The tests are usually performed with special machines into which the person must breathe.

  • Perfusion lung scan. A nuclear medicine procedure that can detect a blood clot in the artery leading to the lung. This procedure can also assess the function of the lungs.

  • Cardiac catheterization. A procedure that evaluates blood flow to the heart muscle, blockage of coronary arteries, congenital heart defects, functioning of the heart valves, and other heart structures. A small catheter is advanced from a blood vessel in the groin or arm through the aorta to the heart.

Treatment for PPH

Specific treatment will be determined by your doctor based on:

  • Your age, overall health, and medical history

  • Extent of the disease

  • Your tolerance for specific medications, procedures, or therapies

  • Expectations for the course of the disease

  • Your opinion or preference

Treatment may include one or more of the following:

  • Medications, including:

    • Anticoagulants. These are used to decrease the tendency of the blood to clot and permit blood to flow more freely

    • Diuretics. These are used to decrease the amount of fluid in the body and reduce the amount of work the heart has to do

    • Drugs. These are used to help lower blood pressure in the lungs and improve the performance of the heart in many patients

    • Calcium channel blocking/vasodilators drugs. These are used to improve the heart's ability to pump blood

  • Supplemental oxygen. Some patients also require supplemental oxygen delivered through nasal prongs or a mask if breathing becomes difficult.

  • Lung or heart-lung transplantation

Treatment Programs

Massachusetts General Hospital understands that a variety of factors influence patients' health care decisions. That's just one reason why we're dedicated to ensuring patients understand their diagnosis and treatment options. Because a single option might not serve all patients, we offer a wide range of coordinated treatments and related services across the hospital. Patients should consult with their primary care doctor or other qualified health care provider for medical advice and diagnosis information.

Select a treatment program for more information:


  • Pediatric Imaging
    The Pediatric Imaging Program at Massachusetts General Hospital Imaging specializes in ensuring the safety and comfort of child patients while providing the latest technology and the expertise of specialized pediatric radiologists.
  • Adult Medicine Imaging
    The Adult Medicine Imaging Program at Massachusetts General Hospital Imaging offers a wide range of diagnostic exams and minimally invasive, image-guided treatments, all provided using leading-edge equipment and interpreted by specialty-trained radiologists.
MassGeneral Hospital for Children

  • Pediatric Pulmonary Medicine
    The Division of Pediatric Pulmonary Medicine at MassGeneral Hospital for Children is a well-established clinical, training and research program. The group provides multidisciplinary comprehensive consultation, diagnostic and management services for a wide array of pulmonary conditions.
Transplant Center

  • Lung Transplant Program
    The Lung Transplant Program at the Massachusetts General Hospital Transplant Center brings together state-of-the-art technology and leading-edge medical and surgical interventions to provide patients with individualized care before and after their lung transplants.
Pulmonary and Critical Care Medicine

  • Cardiopulmonary Exercise Testing Laboratory
    The Cardiopulmonary Exercise Testing (CPET) Laboratory provides comprehensive testing for patients with a variety of heart and lung conditions to determine whether the heart, lungs or skeletal muscles limit exercise capacity.
  • Pulmonary Hypertension and Thromboendarterectomy Program
    The Pulmonary Hypertension and Thromboendarterectomy Program at Massachusetts General Hospital is committed to the state-of-the-art evaluation, treatment and support of patients with all forms of pulmonary hypertension.

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