Pulmonary Hypertension

What is pulmonary hypertension?

Pulmonary hypertension is a serious medical condition which occurs when blood pressure inside the pulmonary arteries is too high. The pulmonary arteries are the vessels that transfer blood away from the heart to the lungs; they are located on the right hand side of the heart.

There are different types of pulmonary hypertension and some are related to underlying medical conditions, usually heart and lung conditions. Pulmonary hypertension can affect people of all ages but some types are more common among particular age groups and there are types that tend to affect women, rather than men. It is estimated that around 2,200 adults are diagnosed with pulmonary hypertension each year in England and Wales.

What causes pulmonary hypertension?

Pulmonary hypertension is caused by changes to the pulmonary arteries; this may involve the arteries becoming blocked or the walls becoming thicker and more rigid. These changes make it more difficult for blood to flow through the arteries and the pressure inside the arteries increases. The World Health Organisation has classified five different types of pulmonary hypertension:

• Pulmonary arterial hypertension.
• Pulmonary hypertension caused by blood clots.
• Pulmonary hypertension related to lung disease and hypoxia.
• Pulmonary hypertension related to left heart disease.
• Other causes.

In some cases, the cause is unknown and this is known as idiopathic pulmonary hypertension. Common causes of PH include:

• Bronchitis.
• Emphysema.

Less common causes include:

• HIV.
• Congenital heart disease.
• Liver disease.
• Vascular or inflammatory conditions.
• Old blood clots in the pulmonary arteries (known as chronic pulmonary thromboembolism).

What are the symptoms of pulmonary hypertension?

Symptoms of pulmonary hypertension include:

• Fatigue.
• Shortness of breath.
• Feeling faint and dizzy.
• Chest pain (also known as angina).
• Rapid heart rate (known as tachycardia).

In some cases, signs do not develop until the illness has become more advanced.

How is pulmonary hypertension diagnosed?

Pulmonary hypertension can be difficult to diagnose because many of the symptoms are similar to other heart and lung conditions. Pulmonary hypertension can be a very serious disease so it is important that you see your GP if you develop any of the symptoms listed above. In many cases, patients do not see their doctor until the symptoms have become more advanced and damage may already have been done by this point. If your GP suspects that you may have pulmonary hypertension, they will carry out tests or refer you to a specialist to have tests in hospital. Tests that may be carried out include:

• An electrocardiogram (commonly known as an ECG).
• Chest X-ray.
• An echocardiogram (this is an ultrasound of the heart).
• Lung function tests.
• Ventilation/perfusion test.
• Exercise tests.

The results of all the tests will be used to reach a firm diagnosis.

Treatment for pulmonary hypertension

It is important to see your GP if you have any of the symptoms associated with pulmonary hypertension. If the condition is left untreated it can cause irreparable damage to the heart and contribute to heart failure. Treatment for pulmonary hypertension usually involves tackling the cause and bringing it under control; this will help to reduce symptoms and prevent further damage to the heart. If you are diagnosed with pulmonary arterial hypertension, you will be referred to a specialist centre; there are seven specialist treatment centres in the UK.

Treatments for pulmonary hypertension include:

• Medication, including anticoagulants and diuretics: anticoagulants are designed to prevent clotting and diuretics are used to remove excess fluid from the body; they cause the individual to urinate more frequently than normal.
• Oxygen (if you have low levels of oxygen in your blood).
• Calcium channel blockers: these are usually used to treat high blood pressure; they make the blood vessels relax and increase the supply of oxygen to the heart.
• Targeted therapy: these are forms of medication which can be used if calcium channel blockers are not suitable.
• Heart lung transplant: this is usually a last resort as it is a very complex procedure and donors are rare; there are as few as 10 procedures carried out each year in the UK.