Pulmonary Hypertension

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General Information About Pulmonary Hypertension

Pulmonary Hypertension Week

What is pulmonary hypertension?

Pulmonary hypertension--or PH--refers to an elevation in pulmonary pressure that can occur in any part of the pulmonary vascular bed. Pulmonary hypertension occurs when the vessels narrow or excessively constrict. This increased the pressure in the lung circulation and causes the right side of the heart (the side responsible for pumping blood into the lungs) to work hard and ultimately can lead to right heart failure and disability and death.
There are several different categories of PH. These are important because they can determine which treatments may be the most effective for a PH patient. There are 5 categories of PH as developed in 2013 by the 5th World Symposium on Pulmonary Hypertension at Nice, France.


The WHO Groups are:

  • WHO Group 1: Pulmonary arterial Hypertension (PAH) including IPAH (idiopathic), HPAP (heritable) and APAP (associated PAH due to other conditions such as connective tissue diseases, toxic exposures, chronic liver disease and HIV).

  • WHO Groups 2-5: Other forms of PH, distinct from PAH pathophysiologically including PH owing to left heart dysfunction, chronic lung diseases (i.e. COPD, IPF, etc), due to chronic pulmonary thromboemboli (clots) and disorders with unclear mechanisms to cause PH.

What causes pulmonary hypertension?

The following are some known causes of PH that correlate to the categories above:

  • Other medical conditions. chronic liver diseases and cirrhosis (Group 1 APAH), rheumatic disorders (Group 1 APAH) such as scleroderma or systemic lupus erythematosis (lupus) and lung conditions (Group 3) including tumors, emphysema, chronic obstructive pulmonary disease (COPD), and pulmonary fibrosis.

  • Heart diseases (Group 2). including aortic valve disease, left heart failure, mitral valve disease, and congenital heart disease, can also cause pulmonary hypertension.

  • Thromboembolic disease (Group 4). Recurrent blood clots that travel to the lung can result in the development of PH.

  • Low-oxygen conditions (Group 3). High altitude living, obesity, and sleep apnea.

  • Genetics (Group 1, IPAH).  PH is inherited in a small number of cases with several different genes identified that increase risk of developing PAH. Knowing that someone in the family had or has PH should prompt you to seek early evaluation should symptoms occur.

  • PH may also be caused by other conditions, and in some cases, the cause is unknown (Group 5).

What are the symptoms of pulmonary hypertension?

  • Shortness of breath
  • Dizziness and/or fainting
  • Chest pain or discomfort
  • Swollen ankles, arms, or legs
  • Feeling tired or worn out
  • Swollen abdomen
  • Rapid, hard, or irregular heartbeat

What tests are needed to diagnose PH?

PH is a complicated problem and patients need to be evaluated and cared for by specialists in PH care. All patients should ask what the experience of their provider has with PH and it they offer comprehensive care.

There are many different tests that need to be performed to identify if PH is present and if so what category that patient is in. these tests include routing history and physical exam, ultrasounds of the heart (echocardiogram), blood tests, tests for blood clots in the lungs, pulmonary function tests, and direct measurements of the pressures in the lung (right heart catheterization). All patients are different and each evaluation is likely a little different.

What are the treatments for pulmonary hypertension?

  • Treatment varies based on the underlying causes, but generally includes:

General supportive therapies

  • Oxygen as needed
  • Pulmonary rehabilitation
  • Diuretics
  • Psychosocial support

Medical therapies

  • Prostacyclin therapies:
    • Intravenous or subcutaneous
    • Inhaled
    • Oral
  • Endothelin receptor antagonists
    • Oral
  • Nitric oxide pathway drugs
    • Oral

Surgical therapies

  • Pulmonary thromboendarterectomy: If present, blood clots in the pulmonary artery may be surgically removed to improve blood flow and lung function.

  • Lung transplantation: Currently, this is the only cure for pulmonary hypertension. Transplantation is reserved for advanced pulmonary hypertension that does not respond to medical therapy. The right side of the heart will generally return to normal after the lung/lungs have been transplanted. About 1,000 lung transplants are performed every year in the United States.

  • Heart/lung transplantation: This type of double organ transplant is very rare but is necessary for all patients who have combined lung and left heart failure.


Four Facts About Pulmonary Hypertension

  1. Pulmonary hypertension is high blood pressure in the arteries going to the lung

  2. If pulmonary hypertension persists or becomes very high, the right ventricle of the heart, which supplies blood to the pulmonary arteries, is unable to pump effectively, and the person experiences symptoms that include shortness of breath, loss of energy, and edema, which is a sign of right heart failure.

  3. A specific type of pulmonary hypertension in which the disease process occurs in the pulmonary arteries themselves is called pulmonary arterial hypertension (PAH).

  4. Pulmonary arterial hypertension has been associated with connective tissue diseases (especially scleroderma), liver disease (portapulmonary hypertension), human immunodeficiency virus (HIV) infection, congenital heart disease, and stimulant drug ingestion.