• Reference Number: HEY-938/2017
• Departments: Pulmonary Hypertension
• Last Updated: 1 September 2017

Introduction

This leaflet offers information about pulmonary hypertension including what it is, how it is diagnosed and how it can be treated.  If you have any questions or concerns after reading this leaflet, please speak to the staff member in charge of your care.

What is pulmonary hypertension?

Pulmonary hypertension is raised blood pressure within the pulmonary arteries.  These are the blood vessels that supply the lungs.  Pulmonary hypertension is very different to the more common type of “high blood pressure”.  It is a serious medical condition that can damage the right side of the heart, making the heart less efficient at pumping blood around the body and getting oxygen to the muscles.

For you to be able to understand pulmonary hypertension better, it is useful to have an understanding of the normal flow of blood through the heart and lungs.  The right ventricle is one of the big pumping chambers of the heart.  It pumps blood that has been circulated around the body through the pulmonary arteries and into the lungs.  In the lungs, the blood picks up oxygen and flows back to the left side of the heart, where it is pumped around the body, beginning the cycle again.

Pulmonary hypertension is where the pressure in the pulmonary arteries becomes higher than normal, causing the walls of the blood vessels to become thicker and less flexible.  Over a period of time the right ventricle also becomes thick and stiff and has to work harder to pump blood through the lungs and the heart.  This means the heart eventually works less efficiently.

What are the symptoms of pulmonary hypertension?

The most common symptoms are:

• shortness of breath
• excessive tiredness/fatigue
• chest pains
• dizziness
• fainting or near fainting episodes
• palpitations or a racing, thumping or fluttering feeling in the chest

These symptoms can occur at rest or during periods of physical activity and can have an impact on a person’s day to day life.

What causes pulmonary hypertension?

There are many different types of pulmonary hypertension.  Often it is linked to diseases such as:

• congenital heart disease (heart disease that you are born with)
• connective tissue disease (for example, scleroderma)
• thromboembolic disease (blood clots in the lungs)
• human immune-deficiency virus (HIV)
• Liver disease (for example, cirrhosis)
• Left heart disease
• Lung disease

Sometimes the cause of pulmonary hypertension is unknown – this is known as idiopathic pulmonary arterial hypertension (IPAH).  In very rare cases, pulmonary hypertension can be inherited.

What can I expect during my consultation and hospital visits?

Your visits to the Pulmonary Hypertension Clinic are very important.  Not only do they ensure that your symptoms are controlled adequately and that you are getting the best possible care, there is also a chance for you to ask questions and/or voice concerns.    The Pulmonary Hypertension Team consists of a range of specialist nurses and doctors who will answer your questions and address any worries you may have.

You may be asked to perform a 6 minute walk test, complete a quality of life questionnaire, have bloods taken and/or have an echocardiogram performed (this is an ultrasound which creates images of the heart).  These tests and investigations help us determine how well you, your heart and lungs are functioning as well as help determine the cause of the pulmonary hypertension.

How is pulmonary hypertension diagnosed?

There are seven designated specialist centres in the United Kingdom that diagnose and treat pulmonary hypertension.  Hull University Teaching Hospitals NHS Trust is a shared care centre, that works in collaboration with The Freeman Hospital in Newcastle.  Information is shared between Hull and Newcastle during the investigation process.

To aid your diagnosis, you may be asked to undergo one or a number of the following tests:

• Blood tests
• Chest X-ray
• Echocardiogram (using ultrasound to create images of the heart)
• Computerised tomography (CT) scan
• Magnetic resonance imaging (MRI) scan
• Lung function tests
• Electrocardiogram (ECG – this tests for problems with the electrical activity of your heart)
• Ventilation/perfusion lung (V/Q) scan (this measures air and blood flow in your lungs)
• Sleep study
• Six-minute walk test (to assess your capacity for exercise)

A confirmed diagnosis of pulmonary hypertension is usually made by carrying out a test called a right heart catheterisation (RHC), which will be performed at Castle Hill Hospital.  A RHC is used to assess how well the heart is pumping and to measure the pressures in the heart and blood circulation of the lungs.  The procedure involves having a small injection of local anaesthetic to numb the skin on your neck.  Occasionally the test can be performed via the groin.  Once the area is numb, a small cut (incision) is made and the doctor will place a catheter (a thin, flexible tube) under your skin and either into the internal jugular vein (if the incision is made to the neck) or the femoral vein (if the incision is made in the groin).  This tube is then guided into your heart.  When the test is finished the tube is gently removed and pressure is applied to the site of insertion to stop any bleeding.  The test takes about 30 minutes to complete.

What are the treatments available?

There is no cure for most types of pulmonary hypertension, however, treatment can be given to help you manage your condition and reduce symptoms. The treatment available depends on the type of pulmonary hypertension that you have.  If your pulmonary hypertension has been caused by an underlying condition, then the condition must also be treated.

Depending on the cause of your pulmonary hypertension, you may have conventional therapies such as oxygen, warfarin (blood thinning medication) and/or diuretics (water tablets).  Alternatively, you may start on more specific therapies, such as:

• Sildenafil
• Tadalafil
• Riociguat
• Bosentan
• Ambrisentan
• Macitentan
• inhaled prostacyclin therapy (Iloprost)
• intravenous prostacyclin therapy (Epoprostenol)

These disease-specific treatments help to relax the arteries in the lungs, allowing blood to flow through these vessels more easily and reducing the blood pressure in your lungs.  These treatments will be discussed with you in more detail if treatment is required.

If your pulmonary hypertension is caused by thromboembolic disease (blood clots in the lungs) you may be offered surgery to remove the clots.  This is called a pulmonary endarterectomy (PEA) and will be discussed with you further if necessary.  In some cases of pulmonary hypertension, disease-specific therapies many not be suitable.  If this is the case the reasons for this will be discussed with you.

Further information is available from the Pulmonary Hypertension Association UK, which publishes a number of free information booklets, downloads, CDs and DVDs for people with pulmonary hypertension, their carers, family and friends.  To learn more visit their website at www.phauk.org.

Contact us:

The Pulmonary Hypertension Team at Castle Hill Hospital consists of: Consultant Respiratory Physicians, Consultant Cardiologists, Specialist Nurses, Specialist Pharmacists, Secretarial support.

For advice or information regarding pulmonary hypertension, please contact the Pulmonary Hypertension Team on telephone number (01482) 622422 (24 hour answer machine).

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