Pulmonary hypertension
What is pulmonary hypertension?
Pulmonary hypertension is a condition where the blood pressure in the pulmonary arteries (the vessels that carry blood from the heart to the lungs) is higher than normal. This puts a strain on the right side of the heart, which has to work harder to pump the blood through.
What causes it?
In a number of cases the cause is unknown (called idiopathic pulmonary arterial hypertension). In other cases pulmonary hypertension may be associated with another disease such as chronic obstructive lung disease (COPD: a long-term condition usually caused by smoking), connective tissue disorders including lupus and scleroderma, sleep apnoea, certain congenital (present from birth) heart defects, HIV infection, and blood clots in the lungs (pulmonary emboli).
Pulmonary hypertension is also associated with some drugs such as certain appetite suppressants ('diet pills'), methamphetamine and cocaine.
Pulmonary hypertension symptoms
Symptoms may include breathlessness, tiredness, fainting and chest pain. To begin with, the symptoms often only occur with exercise, but later they may also occur at rest.
How is it diagnosed?
If your doctor suspects that you may have pulmonary hypertension, they will usually refer you to a centre specialising in this disease for further investigation.
The most important test for the diagnosis of pulmonary hypertension is right heart catheterisation. This involves threading a fine tube up through one of the blood vessels to the heart, so that the pressure in the pulmonary arteries can be measured.
Other tests may be required to look for associated diseases. For example, lung function tests and scans of the lungs and blood vessels may be performed to look for underlying lung disease, or oxygen levels in the blood may be monitored overnight to test for sleep-disordered breathing.
Pulmonary hypertension treatment
The treatment will vary depending on the cause of the pulmonary hypertension.
Medicines may include an anticoagulant (to thin the blood so it flows more easily and is less likely to clot); a diuretic (commonly known as 'water pills', to remove excess fluid and so reduce the work of the heart); and a calcium channel blocker (to relax the blood vessels and so lower the blood pressure).
A number of new medicines have recently become available so that there are now more options for treating pulmonary hypertension. These medicines include:
- vasodilators that open narrowed blood vessels, such as epoprostenol (Flolan), iloprost (Ventavis) and treprostinil (Remodulin);
- medicines that reverse the effect of endothelin, a substance in the walls of blood vessels that causes them to narrow. These are known as endothelin receptor antagonists and include ambrisentan (Volibris) and bosentan (Tracleer); and
- sildenafil (Revatio), which also opens blood vessels in the lungs.
Some patients may also require oxygen therapy if their breathing gets worse. In very severe cases, if medical treatments are not working, a lung transplant may be considered.
Last Reviewed: 12 October 2011
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3. Australian Lung Foundation. Pulmonary arterial hypertension (updated Aug 2011). http://www.lungfoundation.com.au/lung-information/patient-educational-material/fact-sheets/208-pulmonary-arterial-hypertension (accessed Sep 2011).
4. eMIMS prescribing information, Sep 2011.
4. Pulmonary Hypertension Australia. What is pulmonary arterial hypertension? http://www.phaaustralia.com.au/content/what-pulmonary-arterial-hypertension (accessed Sep 2011).
