Portopulmonary hypertension: symptoms, diagnosis and treatment

Pulmonary hypertension is high blood pressure in the blood vessels that supply the lungs, the pulmonary arteries. The NHS explains that the walls of these arteries become thick and stiff and cannot expand as well, which makes the right side of the heart work harder and can gradually weaken it, leading to heart failure.

Portopulmonary hypertension is the form of pulmonary arterial hypertension that occurs together with portal hypertension, which is raised pressure in the portal vein that carries blood from the gut to the liver. The Pulmonary Hypertension Association UK lists portal hypertension, alongside connective tissue disease, HIV infection, congenital heart disease and sickle cell anaemia, as a condition that increases the risk of PAH.

The British Liver Trust lists portal hypertension among the complications and related conditions of cirrhosis, which is scarring of the liver. It says cirrhosis usually takes many years to develop and that most people have no symptoms while it does, so a person may first learn of liver disease when it is found by accident or when they become very unwell.

The symptoms of PoPH are those of pulmonary hypertension in general and can be difficult to tell apart from those of the underlying liver disease. The NHS says symptoms often get worse during exercise and that people with PAH may have no symptoms until the condition is quite advanced.

• Shortness of breath, especially on exertion.

• Tiredness.

• Feeling faint or dizzy.

• Chest pain.

• A racing heartbeat (palpitations).

• Swelling in the legs, ankles, feet or tummy, which can also come from liver disease.

The NHS says pulmonary hypertension can be difficult to diagnose because the symptoms are similar to those of other heart or lung conditions, so there can sometimes be a delay. A GP will ask about symptoms, family history, medicines and other conditions and examine you.

The two main tests are an echocardiogram, a heart scan using sound waves that estimates the pressure in the pulmonary arteries, and right heart catheterisation, in which a thin tube is passed into the pulmonary artery to measure the pressure accurately, carried out in specialist national pulmonary hypertension centres. Other tests include an ECG, chest X-ray, lung function tests, exercise tests, a ventilation-perfusion scan to look for clots and blood tests to rule out other conditions such as thyroid and liver disease.

If pulmonary hypertension is diagnosed, the NHS says it is classified into 4 types depending on how severe your symptoms are, which helps work out the best treatment.

The NHS says pulmonary hypertension cannot be cured but treatment can reduce symptoms and help you manage the condition, and that if the cause is identified and treated early it may be possible to prevent permanent damage to the pulmonary arteries. People are usually referred to a specialist centre; the NHS lists 7 centres in England and 1 in Scotland.

Treatments include medicines that relax the arteries in the lungs, listed by the NHS as endothelin receptor antagonists, phosphodiesterase 5 inhibitors, prostaglandins, soluble guanylate cyclase stimulators and, more recently, biological therapy such as sotatercept, alongside diuretics for fluid and home oxygen if oxygen levels are low. Which treatment is suitable depends on the type, cause and severity.

Liver disease changes the picture. The British Liver Trust says the only cure for cirrhosis is a liver transplant, and not everyone can or needs to have one. In PoPH, the sources used here do not set out how the lung pressures influence transplant decisions, so ask your liver and pulmonary hypertension teams how the two conditions are being managed together.

PoPH is rare and the outlook is serious. A study from the UK National Pulmonary Hypertension Service registry, covering 110 people diagnosed between 2001 and 2010, found survival rates of 85%, 60% and 35% at 1, 3 and 5 years, and concluded that survival remained poor despite targeted therapy and was worse than in idiopathic PAH. The most common causes of portal hypertension in that group were alcohol and hepatitis C, and the average age at diagnosis was 53.

These figures come from a historical group and individual outcomes vary with the liver disease, how early PoPH is found and the response to treatment. The NHS says the outlook for pulmonary hypertension depends on its cause, how quickly it is diagnosed, how advanced symptoms are and any other health conditions, and that the specialist in charge of your care can give detailed information.

The NHS advises seeing a GP if you have symptoms of pulmonary hypertension. 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, because these could be a blood clot in the lungs or a serious heart problem.

Pulmonary Hypertension Association UK offers practical information and support for people living with the condition and their families, including help with emotional wellbeing, exercise, travel, driving and benefits. The NHS notes that pulmonary hypertension can affect your ability to carry out everyday activities, so occupational and benefits advice can matter.