Pulmonary hypertension

What is pulmonary hypertension?

Pulmonary hypertension is high blood pressure in the blood vessels that carry blood from your heart to your lungs, called the pulmonary arteries.

The pulmonary arteries can be affected in different ways, depending on the type of pulmonary hypertension. Most commonly, the heart's right side is forced to work harder to push blood through the lungs, which can weaken the heart over time and cause heart failure.

Pulmonary hypertension symptoms

If you have pulmonary hypertension, you may experience:

• Breathlessness

• Tiredness

• Feeling dizzy or faint

• Chest pains

• A racing heartbeat

• Swollen legs, feet or tummy

Exercising may make symptoms of pulmonary hypertension worse.

Types of pulmonary hypertension

There are several types of pulmonary hypertension – each causes changes to the pulmonary arteries in different ways:

Pulmonary hypertension linked to left heart disease

This is one of the commonest causes of pulmonary hypertension. It happens when there are problems on the heart's left-hand side, forcing the right side to work harder to pump blood to the lungs, increasing blood pressure in the pulmonary arteries.

Pulmonary arterial hypertension

Changes to the smaller pulmonary arteries cause pulmonary arterial hypertension (PAH). The walls of these arteries grow thicker and stiffer, so there is less space for the blood to pass through, increasing blood pressure.

PAH is usually connected to another health condition, like congenital heart problems, connective tissue diseases or sickle cell disease (among many others).

Pulmonary hypertension linked with lung disease or lack of oxygen

Lung diseases, like interstitial lung disease or COPD, are sometimes associated with pulmonary hypertension. A lack of oxygen in the blood, known as hypoxia, is another cause. This narrows the pulmonary arteries, squeezing the blood into a smaller space and causing high blood pressure.

Pulmonary hypertension caused by blood clots

Sometimes, scars from previous blood clots can narrow or block the pulmonary arteries and cause pulmonary hypertension.

When a blood clot blocks a blood vessel that supplies your lungs, it's called a pulmonary embolism.

Pulmonary hypertension diagnosis

It's not always easy to quickly diagnose pulmonary hypertension, as many other heart and lung conditions have similar symptoms. The GP will give you an initial assessment, looking at your symptoms, medical history and any medication you're taking. They may also do a physical examination.

The most specialist tests to diagnose the condition are:

• Echocardiogram – also known as an echo test. This looks at how well your heart is pumping using ultrasound. It can also be used to get a picture of the pressure on your pulmonary arteries.

• Right heart catheterisation – a test that can give an accurate diagnosis using a catheter to measure the blood pressure in the right side of your heart and pulmonary arteries.

Other tests that may be used during diagnosis include:

• Electrocardiogram (ECG) – to check your heart's rhythm and electrical activity

• Chest X-ray – to help identify anything that's causing the breathlessness

• Lung function tests – to assess your breathing

• Blood tests – to help rule out other conditions

• Exercise tests – to monitor your blood pressure, heart rate and oxygen levels while you're active

• Ventilation-perfusion scan – to look for blood clots and measure the air and blood flow in your lungs

After pulmonary hypertension is diagnosed, it will be classified into one of four types, depending on how severe the symptoms are, and how they are affected by physical activities.

Pulmonary hypertension treatment

Although the condition can't be cured, there are lots of treatments to help improve the symptoms, and it's even possible to prevent permanent damage to your pulmonary arteries if the diagnosis is made early enough.

The treatment you have will depend on the type of pulmonary hypertension you have, and whether another condition is causing it or not. You may be referred to one of eight specialist centres around the UK to treat pulmonary hypertension.

Treatment for pulmonary arterial hypertension (PAH)

PAH is treated with pulmonary vasodilators. They open up the pulmonary arteries to lower the blood pressure in your lungs and put less pressure on the right side of your heart.

Other treatments may include diuretics or water tablets, oxygen therapy, anticoagulation medicines to help stop blood clots from forming and pulmonary rehabilitation exercises to help with breathing

Treatment for pulmonary hypertension caused by left heart disease or lung conditions

When pulmonary hypertension is caused by left heart disease or lung conditions, it's a secondary condition. This means it's caused by another heart or lung condition, which is the primary condition that needs treatment.

In these cases, your treatment will be focused on improving the primary condition. This should have a knock-on effect on improving pulmonary hypertension symptoms.

Treatment for pulmonary hypertension caused by blood clots

If blood clots cause the condition, anticoagulation medication can be used to help prevent more blood clots from forming.

Sometimes blood clots can cause scar tissue to form in the pulmonary arteries. There are specialist procedures, including a pulmonary endarterectomy (PEA) and balloon pulmonary angioplasty, to treat this.

Other types of treatment

Other procedures that may be used to treat pulmonary hypertension include:

• Atrial septostomy – a thin, flexible tube called a cardiac catheter is inserted into the heart's chambers or blood vessels. This reduces pressure in the heart's right side, helping the blood flow to the lungs and the heart to pump more effectively.

• Transplant – a lung or heart-lung transplant is rare, but this type of surgery may be used in severe cases.