Pulmonary hypertension: symptoms, causes, tests and treatment

The NHS lists shortness of breath, tiredness, feeling faint or dizzy, chest pain (angina), a racing heartbeat (palpitations) and swelling (oedema) in the legs, ankles, feet or tummy. The symptoms often get worse during exercise, which can limit your ability to take part in physical activities.

The NHS says that if you have pulmonary arterial hypertension (PAH), you may not have any symptoms until the condition is quite advanced. Because breathlessness and tiredness have so many other causes, the NHS notes that the correct diagnosis can sometimes take time.

• Shortness of breath, especially on exertion

• Tiredness and low energy

• Feeling faint or dizzy

• Chest pain or a racing heartbeat

• Swelling of the legs, ankles, feet or tummy

The pulmonary arteries carry blood from the right side of the heart to the lungs, where it picks up oxygen. In pulmonary hypertension, the NHS explains, the walls of these arteries become thick and stiff and cannot expand as well to allow blood through. The reduced blood flow makes it harder for the right side of the heart to pump blood through the arteries. If the right side of the heart has to work harder all the time, it can gradually become weaker, which can lead to heart failure.

It is a different problem from the usual high blood pressure measured in the arm, which relates to the main circulation. Pulmonary hypertension is pressure within the lung circulation, so a normal blood pressure cuff cannot detect it.

The NHS describes it as a rare condition that usually gets worse over time. Left untreated, it may cause heart failure, which can be fatal, so it is important that treatment is started as soon as possible.

The NHS says there are 5 main types of pulmonary hypertension, all involving changes to the pulmonary arteries. The cause decides the treatment, which is why finding it matters.

Within pulmonary arterial hypertension, the NHS lists connective tissue diseases such as scleroderma, congenital heart problems such as a hole in the heart, portal hypertension (high pressure in the vein that takes blood to the liver), HIV, and certain medicines or drugs. A small number of people develop PAH without any other medical condition (idiopathic PAH), and in very rare cases it can be inherited.

The NHS adds that left heart problems are thought to be one of the most common causes overall, and lists less common causes such as sarcoidosis, vasculitis, thyroid disease, glycogen storage disease, sickle cell disease, thalassaemia, chronic kidney disease and compression of lung blood vessels by a tumour.

The NHS advises seeing a GP if you have any symptoms of pulmonary hypertension. The GP may ask about your symptoms and medical history and may carry out a physical examination.

The NHS says to call 999 or go to A&E if you get sudden chest pain that does not stop after a few minutes, or heart palpitations with chest pain, shortness of breath or feeling faint. It says these could be a sign of another condition such as a blood clot in the lungs or a serious heart problem.

The NHS says a GP may ask about your symptoms and how they affect your life, your family history (pulmonary arterial hypertension can rarely run in families), the medicines you take and other medical conditions, and may listen to your heart and lungs and check for swelling in the legs or ankles.

The two main tests are an echocardiogram and right heart catheterisation. The echocardiogram uses high-frequency sound waves to create an image of the heart, estimates the pressure in the pulmonary arteries and tests how well both sides of the heart are pumping. Right heart catheterisation passes a thin, flexible tube through a vein in the neck, arm or groin into the pulmonary artery to measure pressure accurately, and the NHS says it is carried out in specialist national pulmonary hypertension centres.

Other tests the NHS lists include an ECG, a chest X-ray, lung function tests, exercise tests, a ventilation-perfusion scan to look for clots, and blood tests to rule out conditions such as thyroid and liver disease. Once diagnosed, the NHS says the condition is classified into 4 types by how severe symptoms are, to help work out the best treatment.

The NHS says the treatment depends on the type, the cause and the severity of symptoms. If another heart or lung problem is the cause, treatment focuses on that underlying condition. If blood clots in the pulmonary arteries are the cause, anticoagulant medicines may be offered, and possibly an operation called pulmonary endarterectomy.

General treatments include anticoagulants such as warfarin, diuretics (water tablets), home oxygen therapy if blood oxygen is low, and digoxin. There are also specialist medicines that relax the arteries in the lungs and reduce the pressure in them, which may slow progression and help control symptoms. Some people need surgery or procedures; the NHS lists pulmonary endarterectomy, balloon pulmonary angioplasty, atrial septostomy and, in severe cases, lung or heart-lung transplant, which it says is rarely used because effective medicine is available.

The NHS says the outlook varies depending on what is causing the condition, how quickly it is diagnosed, how advanced your symptoms are and whether you have another underlying health condition. The specialist in charge of your care can give more detailed information for your situation.

The NHS notes that having pulmonary hypertension can affect your ability to carry out everyday activities. The charity Pulmonary Hypertension Association UK offers practical information and support for people living with the condition and for their friends and families.

In the NHS system, suspected pulmonary hypertension is investigated by a GP, a cardiologist or respiratory specialist, and confirmed cases are referred to one of the designated specialist centres. The initial echocardiogram may be done locally.

Private cardiology and respiratory consultations can help with early assessment, but the specialist medicines and procedures described above are delivered through the designated specialist centres, so many people move between local, private and specialist services.