Treating pulmonary hypertension

If you havepulmonary hypertension caused by another underlying condition, your treatment will focus on tackling it.

If the cause is identified and treated early, it may be possible to prevent permanent damage to your pulmonary arteries (the blood vessels that supply your lungs).

If you have pulmonary arterial hypertension (PAH) , you will be referred to acentre thatspecialises in treating this form of the condition. There are seven centres in England and one in Scotland. They are:

• Great Ormond Street Hospital (for children), London
• Hammersmith Hospital , London
• Royal Brompton Hospital , London
• Royal Free Hospital , London
• Papworth Hospital NHS Trust , Cambridgeshire
• Freeman Hospital , Newcastle
• Golden Jubilee National Hospital , Glasgow
• Royal Hallamshire Hospital , Sheffield

There are anumber of medicines used to treat PAH and other types of pulmonary hypertension.

You will usually be started on conventional therapy or background therapy (see below),before your treatment is changed or increased as necessary. Further treatment is called targeted therapy.

Background therapy

Background therapy may include:

• Anticoagulant medicines
• diuretics(medicines that increase urine output)
• oxygen
• digoxin

These are explained in more detail below.

Anticoagulant medicines

PAH can increase your risk of developing venous thromboembolism, which is where a blood clot forms in a vein and breaks free.

The clot can become trapped in a blood vessel, blocking the vessel and the blood supply.If ablood clot blocks a blood vessel supplying your lungs, it is knownas a pulmonary embolism .

Anticoagulant medicines reduce the blood's ability to clot. Taking anticoagulants, such as warfarin , may reduce your risk of developing venous thromboembolism.

Anticoagulants can cause side effects, such as increased bleeding, so you will need to be carefully monitored while taking them.

Diuretics

Diuretics are used to remove excess fluid from the body by increasing the production and flow of urine.

In pulmonary hypertension,diureticsmay be usedto treat fluid retention and swelling ( oedema ). This can occur if the condition starts to cause heart failure , where yourheart struggles to pump blood around your body.

Diuretics used include:

• furosemide
• amiloride
• spironolactone

Diuretics can cause side effects such as:

• mild tummy upset
• postural hypotension where your blood pressure drops suddenly after moving, which can make you feel dizzy after you stand up
• a dry mouth
• rashes
• headaches
• confusion

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.

Oxygen

If you have low oxygen levels in your blood, you may need to be treated with oxygen. This involves inhaling air that containsa higher concentration of oxygen than normal. This will increase the amount of oxygen in your blood and should help to improve your symptoms.

Read information about home oxygen treatment .

Digoxin

If pulmonary hypertension leads to heart failure, you may be treated with digoxin. Digoxin increases the strength of your heart muscle contractions and can slow down your heart rate.

Targeted therapy

Calcium channel blockers

Calcium channel blockers are medicines usually used to treat high blood pressure (hypertension) and angina (pain in the heart caused when the blood supply to the heart's muscles is restricted).

Calcium channel blockers work by relaxing your artery wall muscles. This widens your arteries and reduces your blood pressure.

Nifedipine and diltiazem are two possible calcium channel blockers that may be prescribed.

Calcium channel blockers may work for people with idiopathic PAH (where there is no known cause). However, the effect in other types of PAH is less clear.

You will need a vasoreactivity test to find whether calcium channel blockers are suitable for you. This involves taking the medication and then checking whether the blood pressure in your pulmonary arteries has fallen. If it has, you may be prescribed calcium channel blockers.

If calcium channel blockers are not suitable for you, or are no longer effective, further treatments can be used. These are called targeted therapies and include the following medicines:

• bosentan
ambrisentan
• macitentan
• sildenafil
• tadalafil
• iloprost
• veletri
• epoprostenol
• riociguat

Some of these are described below.

Bosentan

Bosentantablets may helpto improve your symptoms and ability to exercise. Two tablets are usually taken twice a day (in the morning and evening), although the dosage can sometimes vary.

You may need to spend a day in hospital when you start treatment. If your condition has not improved after8-16 weeks, or if ithas got worse, you may be given additional treatment as well as, or instead of, bosentan.

Bosentan can increase levels of some enzymes in the liver, which can causeliver damage.It may also cause anaemia in some people.As a result, your liver will need to be monitored once a month for as long as you are taking bosentan.

Fluid retention for example,leg swelling has been reported in up to 30% of patients using bosentan.

Bosentan may not be suitable for you if you:

• already have a problem with your liver
• are takingciclosporin (a medicine that suppresses your immune system)
• are pregnant

Women who could get pregnant must use contraception if they are taking bosentan.

This type of medicationcan interact with hormonal contraception, such as the combined contraceptive pill , so it's better to use an alternative method, such as condoms .

Ambrisentan and macitentan

These act insimilar waysto bosentan, but only need to be taken once a day. The side effects are also similar, and monthly blood tests will berequired to exclude liver abnormalities and anaemia.

Sildenafil

Sildenafiltablets are usually taken three times a day to improve your ability to exercise.

You may need to spend a day in hospital when you start treatment, before returning home and carrying 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 liver problem
• have recently had a heart attack or stroke
• have severe low blood pressure (hypotension)
• have certain eye conditions

Tadalafil

Tadalafil acts in exactly the same as sildenafil, but only needs to be taken once a day. Its side effects are also similar to sildenafil.

Iloprost

Iloprostis inhaled through a nebuliser. This is a device that turns the liquid medicine into a mist you breathe in to your lungs. You may need to take iloprostsix toninetimes a day. Each dose can take up to 10 minutes to inhale.

You will need to stay in hospital for up to three days, so that you can be trained in using the nebuliser, and to monitor your response. After this, you should be able to return home and continue taking the medicine.

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 recentlyhad 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

Epoprostenol (full name: epoprostenol sodium) may be used if you do not respond to other treatments or if your condition is severe.

Epoprostenol needs to be given through an intravenous infusion, where a constant drip of medicine passes through a narrow tube into a vein in your arm or chest.

Treatment with epoprostenol will begin in hospital. It may takeone to twoweeks 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 administer the medicine yourself at home.

Once you have started taking epoprostenol, you may not be able to stop. Coming off epoprostenol may cause your pulmonary hypertension to rapidly return, so you may need to take the medication for the rest of your life. This is why epoprostenol is considered last, after other treatments have not worked or 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).

Lung or heart-lung transplant

A transplant may be an option in cases of severe pulmonary hypertension. It is a major surgical procedure that involves transplanting healthy lungs or a heart and lungs from a donor into a recipient.

However, less than 200 lung and heart-lung transplants arecarried out each year in the UK, due to a shortage of donors.

and heart-lung transplants .

Pulmonary Hypertension Association UK

Pulmonary Hypertension Association UK is a charity for people with pulmonary hypertension.

The website contains further information and adviceon all aspects of pulmonary hypertension, including living with pulmonary hypertension and support for family and friends .