Pulmonary Hypertension (PH) is the name given to describe high blood pressure within the lungs, (pulmonary means that it is to do with the lungs and hypertension means high blood pressure). Pulmonary hypertension affects two in every million people. PH affects people of all ages and from all ethnic backgrounds.

What causes pulmonary hypertension and who can get it?

Pulmonary hypertension is when high pressure in the lungs causes the blood vessels in the lungs to become very narrow.  They can sometimes become blocked. This means that the heart has to work extra hard to pump the blood through the narrowed vessels. Eventually, the heart enlarges with the strain and pumps less efficiently. Therefore the body receives less oxygen.

There are two types of pulmonary hypertension:

• Idiopathic Pulmonary Arterial Hypertension (IPAH) is the term used either when there is no explanation for the pulmonary hypertension or when a genetic abnormality has been found which has caused the disease. The genetic abnormality rarely occurs in families.
• Associated or Secondary pulmonary hypertension occurs when the condition is associated with another illness such as scleroderma, the CREST syndrome and some forms of congenital heart disease.

What are the signs and symptoms of pulmonary hypertension?

People who have pulmonary hypertension may:

• Feel out of breath very easily
• Have chest pain
• Faint or feel dizzy
• Feel very tired all the time
• Have swollen ankles, legs or stomach

These symptoms are present in many illnesses and so it can be quite difficult to diagnose pulmonary hypertension.

How is it diagnosed?

Several tests are needed to make the diagnosis and to determine how well your heart and lungs are working. Such tests include:

• Echocardiogram (Echo) – to scan your heart and see how it is working
• Electrocardiogram (ECG) – to measure your heart’s electrical activity
• Cardiac catheterisation – to measure the pressure in your heart and the resistance to blood flow through the lungs
• A walk test – to see how your body can cope with exercise

How is it treated?

There is no cure for pulmonary hypertension but there are now many new treatments that can help a lot.  There has been lots of research into the illness during the past few years and there is still a huge amount of research in progress. Treatments include:

• Medicines to reduce the pressure in the lungs
• Medicines that will help to stop the vessels from getting narrower
• Medicines to help the heart pump better
• Medicines to prevent blood clots in the lungs so that blockages are less likely

If these treatments do not help sufficiently, it’s possible you may have to have a lung transplant.

Looking forward

Pulmonary hypertension is a serious, incurable illness. However, there have been major advances in treating the condition during the past 5-10 years and the future is promising.

For more help and information

Please visit the Pulmonary Hypertension Association UK or talk to your doctor.
 
Last reviewed by Great Ormond Street Hospital: 7 February 2007

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