Pulmonary arterial hypertension living

Overview

Pulmonary arterial hypertension (PAH) is a rare but serious condition where the blood pressure in the arteries that carry blood from your heart to your lungs is too high. This makes the right side of your heart work much harder to pump blood, which can weaken the heart over time. PAH is a long-term condition, but with the right care, many people live active and full lives.

No, PAH is rare. It affects only a small number of people each year, and doctors often need several tests to confirm it.

PAH can affect anyone at any age, but it is more common in women and usually develops between the ages of 30 and 60. Some people inherit it from a family member, and others may develop it because of another medical condition.

Symptoms

Common symptoms

• Shortness of breath during everyday activities such as climbing stairs
• Tiredness and low energy
• Chest pain or tightness, especially during activity
• Dizziness or fainting spells
• Swelling in the ankles, legs, or belly
• Heart palpitations (feeling like your heart is racing)
• Bluish or greyish lips and skin

Symptoms in children

• Poor feeding or trouble gaining weight
• Breathlessness while feeding or playing
• Unusual tiredness or excessive sleeping
• Irritability or fussiness without a clear reason
• Bluish lips or skin

Symptoms in older adults

• Shortness of breath with activity that may be mistaken for normal aging
• Leg swelling and fatigue
• Difficulty walking or keeping up with others
• Feeling of faintness or falls

Causes

Main causes

• The exact cause is often unknown — this is called idiopathic PAH.
• Some cases run in families and are caused by inherited gene changes.
• It can be linked to other conditions, such as connective tissue disease, liver disease, congenital heart disease, or HIV.
• In rare cases, certain medicines or toxic substances may trigger PAH.

Risk factors

• Being female
• Having a family history of PAH
• Being aged 30 to 60
• Having certain other conditions, including autoimmune or liver diseases
• Using certain illegal drugs or appetite-suppressant medicines (ask your doctor for guidance)

When to see a doctor

See a doctor urgently if:

• Call your local emergency number if you have severe chest pain, faint, cough up blood, or cannot catch your breath even at rest.

Book a routine appointment if:

• See your GP or healthcare provider if you have lasting breathlessness, tiredness, dizziness, or ankle swelling that is new or getting worse.
• If you have a known family history of PAH, talk to your doctor about check-ups even before symptoms appear.

Diagnosis

To diagnose PAH, your doctor will ask about your medical history, listen to your heart, and do a physical exam. If they suspect PAH, they will refer you to a specialist centre for tests that measure the pressure in your lungs and rule out other causes.

Tests that may be done

• Blood tests
• Electrocardiogram (ECG) to check your heart's rhythm
• Chest X-ray
• Echocardiogram (ultrasound of the heart)
• Right heart catheterisation (a thin tube used to measure blood pressure directly in the lung arteries)
• Exercise tests such as the 6-minute walk test

What to expect at your appointment

Diagnosis can take time and often involves several hospital visits. Some tests are quick and painless, while others require staying in hospital for a day. Your specialist team will explain each step and support you throughout the process.

Treatment

PAH is not curable, but it is very treatable. The aim is to reduce symptoms, slow the disease, and help you stay active as long as possible. Treatment is usually led by a specialist team and tailored to your personal situation.

Self-care at home

• Take all prescribed medicines exactly as directed by your doctor.
• Stay active within comfortable limits, but avoid very strenuous exercise or heavy lifting.
• Monitor your weight daily to spot fluid build-up early.
• Avoid smoking, vaping, and illegal drugs.
• Talk to your doctor before planning a pregnancy.
• Keep all follow-up appointments with your PH team.

Medical treatments

There are many treatment options. Some are medicines taken by mouth, others are inhaled through a nebuliser, and some are given as injections under the skin or through a vein. These treatments help relax and widen the blood vessels in your lungs, lowering pressure and making it easier for your heart to pump. You may also be given oxygen therapy and medicines that remove extra fluid from your body. Your doctor will discuss the best plan for you.

When is surgery considered?

If medicines are no longer working well and PAH becomes very severe, a lung transplant or heart-lung transplant may be considered. This is a major operation, but it can be life-saving for some people. Another procedure, called atrial septostomy, opens a small hole between the upper heart chambers to reduce pressure — but it is only used in selected cases.

Living with this condition

Living with PAH means learning to pace yourself and listening to your body. Plan rest breaks during the day and consider using a mobility aid if you get short of breath. Keep a daily record of your symptoms so you can share changes with your care team.

Lifestyle tips

• Eat a heart-healthy diet with less salt to reduce swelling.
• Maintain a healthy weight and avoid crash diets.
• Do not smoke and avoid second-hand smoke.
• Avoid alcohol and drugs that can strain the heart.
• Take precautions in extreme temperatures, as both heat and cold can worsen symptoms.
• Travel with a travel oxygen plan if you use oxygen — discuss this with your team.

Diet and exercise

A low-salt, balanced diet can help control fluid build-up. Exercise is valuable, but it needs to be done safely. A supervised pulmonary rehabilitation programme is ideal, as it teaches you exercises that match your condition. In general, aim for gentle activities such as walking, stretching, or cycling, and avoid heavy lifting or intense sports.

Mental health and emotional wellbeing

Living with a long-term condition can cause anxiety, stress, or low mood. These feelings are normal. It is important to talk about them — with your doctor, a counsellor, or a trusted friend. Taking care of your mental health is as important as treating PAH itself.

Prevention

Most cases of PAH cannot be completely prevented. However, you can lower your risk of some forms by avoiding smoking and illegal drugs, staying physically active, and keeping other health conditions well managed. If PAH runs in your family, talking to a genetic counsellor can help you understand your risk.

Vaccines

Keeping your vaccinations up to date is very important. Vaccines for flu, COVID-19, and other respiratory infections can lower your chances of getting seriously ill and making PAH worse. Ask your pharmacist or GP about which vaccines you need.

Screening programmes

If you have a family history of PAH, regular check-ups with an echocardiogram may help catch changes early. For people with conditions linked to PAH, yearly check-ups may be useful. Your doctor can help you decide on the right screening schedule.

Complications

If left untreated

• Right heart failure, where the heart becomes too weak to pump blood properly
• Abnormal heart rhythms (arrhythmias)
• Blood clots in the lung arteries
• Bleeding from the lungs
• Serious complications during pregnancy
• Life-threatening loss of heart function

Long-term outlook

A PAH diagnosis can feel frightening, but there is real hope. With modern treatments and close specialist care, many people live for years — and sometimes decades — after diagnosis. You can have a meaningful, active life. Your care team will work with you every step, helping you adjust, thrive, and plan for the future.

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