Pulmonary Hypertension

Overview

Pulmonary hypertension is high blood pressure in the arteries of your lungs. These arteries carry blood from the heart to the lungs to pick up oxygen. When they become narrow or stiff, the heart has to work harder to pump blood through them. Over time, this can weaken the heart and make it hard to breathe.

Pulmonary hypertension is uncommon. In the UK, it is estimated that about 7,000 people are living with it. Many people have symptoms for a long time before it is found.

Pulmonary hypertension can affect people of all ages, but it is more common in adults over 40. Some types are more common in women. Children can also get it, but this is rare.

Symptoms

Common symptoms

• Shortness of breath during everyday activity
• Tiredness and fatigue
• Chest pain or pressure
• Dizziness or fainting
• Swelling in the ankles, legs or abdomen
• Palpitations (feeling the heart racing)

Symptoms in children

• Poor appetite
• Slow growth
• Tiredness
• Shortness of breath during play

Symptoms in older adults

• Breathlessness that is put down to ageing
• Confusion or reduced alertness
• Leg swelling
• Fatigue

Causes

Main causes

• No known cause (called idiopathic)
• A genetic change that runs in families
• Lung diseases such as COPD or sleep apnoea
• Left-sided heart disease
• Blood clots in the lungs
• Connective tissue diseases like scleroderma

Risk factors

• Family history of pulmonary hypertension
• Another condition that causes high pressure in the lung arteries
• Smoking
• Living at high altitude
• Use of certain appetite-suppressant medicines or recreational drugs

When to see a doctor

See a doctor urgently if:

• Call your local emergency number if you have severe chest pain, faint, suddenly cannot breathe, or cough up blood.

Book a routine appointment if:

• Make an appointment with your GP if you are short of breath with everyday activities, feel unusually tired, notice swelling, have dizziness, or have chest pain that comes and goes.

Diagnosis

Your doctor will ask about your symptoms, medical history, and family history. They will listen to your heart and lungs and may do simple tests first. If they think you might have pulmonary hypertension, they will refer you to a specialist team at a specialised centre.

Tests that may be done

• Blood tests
• Chest X-ray
• Electrocardiogram (ECG)
• Echocardiogram (ultrasound of the heart)
• Breathing tests (spirometry)
• Right heart catheterisation (a thin tube to measure pressure in the lung arteries)

What to expect at your appointment

Diagnosis can take time. You may need to visit a specialist centre for some tests. Most tests are painless and done as an outpatient. A right heart catheter needs a short hospital stay. After tests, your team will explain what they found and work with you to make a care plan.

Treatment

There is no cure for most pulmonary hypertension, but treatment can relieve symptoms, slow the condition, and improve quality of life. Your specialist will tailor treatment to the cause and your individual needs.

Self-care at home

• Pace yourself and plan your energy
• Avoid heavy lifting and straining
• Keep active within your limits
• Weigh yourself regularly if your team advises, as sudden weight gain can be a sign of fluid buildup
• Avoid smoking
• Talk to your doctor before flying or going to high altitude

Medical treatments

Treatments include oxygen therapy, medicines that relax blood vessels or reduce fluid buildup, and sometimes procedures. Your specialist team will discuss the options with you. Never start or stop any medicine without medical advice. Do not take over-the-counter cold or weight-loss medicines without checking with your pharmacist or doctor first.

When is surgery considered?

In severe cases, surgery may be considered, such as a heart-lung transplant. This is only for certain patients and requires careful evaluation by a transplant team.

Living with this condition

Living with pulmonary hypertension means managing your energy and staying in tune with your body. Plan rest breaks, keep warm, and avoid sudden changes in temperature. Ask for help when you need it.

Lifestyle tips

• Stay as active as your doctor recommends — gentle walking is often fine
• Eat a heart-healthy diet with less salt
• Avoid smoking and second-hand smoke
• Avoid pregnancy unless your specialist has discussed it — it can be risky
• Manage stress with relaxation or breathing techniques

Diet and exercise

A balanced diet low in salt can help reduce fluid retention. Work with a dietitian if possible. A supervised exercise programme, such as cardiac rehabilitation, can improve fitness without overtaxing your heart.

Mental health and emotional wellbeing

Living with a long-term condition can cause anxiety, low mood, or stress. It is normal to feel this way. Talk to your healthcare team about mental health support. If you have thoughts of self-harm, seek help immediately or contact a crisis line.

Prevention

You cannot always prevent pulmonary hypertension, especially if it is genetic. But you can reduce some risks by staying physically active, maintaining a healthy weight, not smoking, and treating other heart and lung conditions early.

Vaccines

Keep up to date with flu, COVID-19, and pneumonia vaccines. Ask your doctor or pharmacist which ones are recommended for you.

Screening programmes

Screening is not routine, but if you have a family history of pulmonary hypertension, talk to your doctor about monitoring your heart and lungs.

Complications

If left untreated

• Right heart failure (the heart becomes too weak to pump)
• Irregular heart rhythms
• Blood clots in the lung arteries
• Fainting or collapse
• Reduced ability to do daily tasks

Long-term outlook

With early diagnosis and the right treatment, many people with pulmonary hypertension live active and full lives. The outlook varies from person to person, but treatment has improved a great deal. Your team will help you manage risks and adjust your care over time.

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