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 can be seen in association with increased pressure as a result of a decrease in the luminal diameters of small pulmonary arteries.

Pulmonary arterial hypertension--or PAH--is a progressive disease defined by elevated pulmonary artery pressure and pulmonary vascular resistance leading to right ventricular failure and premature death.PH is divided into five WHO groups. Developed in 2013 by the 5th World

Symposium on Pulmonary Hypertension at Nice, France, the WHO group system includes:

  • WHO Group 1: iPAH and several subtypes
  • WHO Groups 2-5: Other forms of PH, distinct from PAH pathophysiologically


What causes pulmonary hypertension?

The following are some known causes of pulmonary hypertension:

  • Liver diseases, rheumatic disorders, lung conditions. Pulmonary hypertension also can occur as a result of other medical conditions, such as chronic liver disease and liver cirrhosis; rheumatic disorders such as scleroderma or systemic lupus erythematosus (lupus); and lung conditions, including tumors, emphysema, chronic obstructive pulmonary disease (COPD), and pulmonary fibrosis.
  • Certain heart diseases. Heart diseases, including aortic valve disease, left heart failure, mitral valve disease, and congenital heart disease, can also cause pulmonary hypertension.
  • Thromboembolic disease. A blood clot in a large pulmonary artery can result in the development of pulmonary hypertension.
  • Low-oxygen conditions. High altitude living, obesity, and sleep apnea can also lead to the development of pulmonary hypertension.
  • Genetics. Pulmonary hypertension is inherited in a small number of cases. Knowing that someone in the family had or has pulmonary hypertension should prompt you to seek early evaluation should symptoms occur.
  • Pulmonary hypertension may also be caused by other conditions, and in some cases, the cause is unknown.

What are the symptoms of pulmonary hypertension?

The most common PAH symptoms are:

  •   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 are the treatments for pulmonary hypertension?

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

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 PAH

  1. Women are up to four times more likely than men to be diagnosed with pulmonary arterial hypertension.

  2. The median time between symptom onset and diagnosis is 14 months. This time has not changed in the past 20 years. 

  3. Nearly 75% of PAH patients are in the later phase of the disease by the time they are diagnosed. By this point they are experiencing significant limitations in ordinary physical activity and treatments are less likely to be effective.

  4. Many patients will see three or more different physicians over a three year period before they are properly diagnosed with PAH.