Understanding Pulmonary Hypertension

Pulmonary hypertension, or PH, is a simplified name for a complex health problem: high blood pressure in the lungs. The name comes from pulmonary, or having to do with the lungs, and hypertension, which means high blood pressure. It is a disease that affects people of all ages and ethnic backgrounds.
One thing that most types of PH have in common is that they are often not recognized until they are quite advanced. As a result, many people may have PH without knowing it - so higher awareness and earlier diagnosis can help people get earlier treatment. There are several different kinds of PH.
PH starts when the small vessels that supply blood to the lungs constrict, or tighten up. It is more difficult for blood to get through to the lungs, and the heart must pump harder to overcome the resistance. As time passes, scarring (or fibrosis) of the vessels makes them stiffer and thicker, and some may be completely blocked. The extra stress causes the heart to enlarge and become less flexible. Less and less blood is able to flow out of the heart, through the lungs, and into the body, and more and more symptoms start to show.
PH is often not diagnosed in a timely manner because its early symptoms can be confused with those of many other conditions. Some of the symptoms of PH include:
  • Breathlessness or shortness of breath
  • Feeling tired all the time
  • Dizziness, especially when climbing stairs or upon standing up
  • Fainting - often the symptom that brings people to their doctors
  • Swollen ankles and legs
  • Chest pain, especially during physical activity
To establish a diagnosis of PH, a series of tests are performed that show how well a person's heart and lungs are working. These tests may include:

  • Echocardiogram
  • Electrocardiogram (ECG)
  • Right heart catheterization
  • Six-minute walk test
  • Assessment of function in tasks of daily living
If these tests show that a person definitely has PH, other kinds of diagnostic tests may be ordered to aid in identifying the cause, including:
  • A computed tomography (CT or CAT) scan to rule out a pulmonary embolism or lung disease
  • A pulmonary function test to rule out obstructive lung disease
  • A formal sleep study to rule out sleep apnea
  • Laboratory tests to rule out hepatitis, collagen disease, HIV, or other conditions
When no cause can be found, the diagnosis is primary pulmonary hypertension (PPH).

From the Pulmonary Hypertension Association.

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