If your pulmonary hypertension is caused by an underlying condition, your treatment will focus on tackling it. If the cause is treated early, it may be possible to prevent permanent damage to your pulmonary arteries (the blood vessels supplying your lungs).
If you have pulmonary arterial hypertension (PAH), you will be referred for treatment at a centre that specialises in PAH. There is one centre in Scotland at the Golden Jubilee National Hospital in Clydebank.
A number of different medicines can be used to treat PAH and other types of pulmonary hypertension. You will usually be started on conventional therapy or background therapy, and your treatment will then be changed or increased as necessary. Further treatment is called targeted therapy.
Background therapy includes:
- anticoagulant medicines
These are explained in more detail below.
PAH can increase your risk of venous thromboembolism, which is when a blood clot forms in a vein and then breaks free. The clot can become trapped in a blood vessel, blocking the vessel and the blood supply. If this blocks one of the blood vessels supplying your lungs, this is called a pulmonary embolism.
Anticoagulant medicines reduce the ability of the blood to clot. Taking anticoagulants, such as warfarin, may therefore reduce your risk of venous thromboembolism.
These medicines can cause side effects, such as increased bleeding, so you will be carefully monitored while taking them.
Diuretic medicines are used to remove excess fluid from the body by increasing the production and flow of urine. Diuretics may be used in pulmonary hypertension to treat any fluid retention and swelling (oedema). This can occur if pulmonary hypertension starts to cause heart failure (when your heart struggles to pump blood around your body).
Possible diuretics that may be used include:
Diuretics can cause some side effects, such as:
- mild tummy upset
- postural hypotension – when your blood pressure drops suddenly after movement – for example, feeling dizzy after standing up
- a dry mouth
Diuretics can also disrupt your levels of electrolytes (minerals in your blood, such as potassium) and affect your kidney function. Because of this, your kidneys and blood will be monitored while you are taking diuretics.
If you have low oxygen levels in your blood, you may need to be treated with oxygen. This involves inhaling air that has a higher amount of oxygen in it than normal. This increases the amount of oxygen in your blood and should improve your symptoms.
If your pulmonary hypertension leads to heart failure, you may be treated with digoxin. This increases the strength of your heart muscle contractions and can slow down your heart rate.
Calcium channel blockers
Calcium channel blockers are medicines normally used to treat high blood pressure (hypertension). They work by relaxing the muscles that make up the walls of your arteries. This widens your arteries and reduces your blood pressure.
Possible calcium channel blockers include:
Calcium channel blockers may work for people with idiopathic PAH (PAH with no known cause), but the effect in other types of PAH is less clear.
You will need a vasoreactivity test to find out if calcium channel blockers will be suitable for you. This involves taking some of the medication and then checking the blood pressure in your pulmonary arteries to see if it has fallen. If it has, you may be prescribed calcium channel blockers.
If calcium channel blockers were not suitable for you, or if they are no longer effective, further treatments can be tried. These are called targeted therapies and include the following medicines:
These are explained in more detail below.
Bosentan tablets may be taken to improve your symptoms and your ability to exercise. You usually take two tablets twice a day (in the morning and evening), although the dose can vary.
You may spend a day in hospital starting treatment and should then be able to return home and carry on taking the medicine. If after 8-16 weeks your condition has not improved (or has got worse), you may be given additional treatment as well as or instead of bosentan.
Bosentan can increase the levels of some enzymes in the liver, which can lead to liver damage. Enzymes are proteins that speed up and control chemical reactions in the body, such as digestion. Because of this, your liver will need to be monitored once a month for as long as you are taking bosentan.
Bosentan will not be suitable for you if you:
- already have a problem with your liver
- are taking ciclosporin (a medicine that suppresses your immune system)
- are pregnant
Women who could get pregnant must use contraception if they are taking bosentan. Bosentan can interact with hormonal contraception, such as the combined contraceptive pill, so it is better to use an alternative method, such as condoms.
Sildenafil tablets are usually taken three times a day to improve your ability to exercise.
You may spend a day in hospital starting treatment and should then be able to return home and carry on taking the medicine. How long you can take sildenafil for will depend on your condition. If at any time your condition gets worse, you may be given additional treatment as well as or instead of sildenafil.
Sildenafil may not be suitable for you if you:
- are also taking medicines or drugs that contain nitrates – organic nitrates are often used to treat angina, and amyl nitrate is a recreational drug commonly known as 'poppers'
- have a severe problem with your liver
- have recently had a heart attack or stroke
- have severe low blood pressure
- have certain eye conditions
Iloprost is inhaled through a nebuliser. This is a device that turns the liquid medicine into a mist that you breathe into your lungs. You may need to take iloprost around six to nine times a day. Each dose can take up to 10 minutes to inhale.
You will need to stay in hospital for up to three days to be trained in using the nebuliser and to monitor your response. After this time, you should be able to return home and keep taking the medicine yourself.
How long you need to take iloprost for will depend on your condition. You may need to continue taking it, possibly alongside other treatments, until your condition worsens and you need epoprostenol (see below).
Iloprost may not be suitable if:
- you have a peptic ulcer
- you have recently had a heart attack or stroke
- your pulmonary hypertension is caused by pulmonary veno-occlusive disease (a rare condition that causes high blood pressure in the lungs)
- your pulmonary hypertension is unstable, with advanced right heart failure
Epoprostenol – its full name is epoprostenol sodium – may be used if you do not respond to other treatments. Epoprostenol needs to be given through an intravenous infusion, which means a constant drip of medicine goes through a narrow tube into a vein in your arm.
Treatment with epoprostenol will begin in hospital. It may take one or two weeks to find the correct dose for you. After this time, it is possible, with special training and equipment, for you or your carer to learn how to take this medicine yourself at home.
Once you have started taking epoprostenol, you may not be able to stop. Coming off epoprostenol can cause your pulmonary hypertension to rapidly return, so you may need to take epoprostenol for the rest of your life. This is why epoprostenol is considered last, after other treatments have not worked or if your condition has got worse.
Epoprostenol may not be suitable for you if you have heart failure caused by a problem with the left side of your heart, or if you have or develop pulmonary oedema (fluid on the lungs).
For severe pulmonary hypertension, a heart-lung transplant may be a treatment option. This involves transplanting the healthy heart and lungs from a donor into you. However, because of a shortage of donors, these are rarely carried out. There are probably fewer than 10 heart-lung transplants a year in the UK.
Read more on heart-lung transplants.