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What is pulmonary hypertension?
Pulmonary hypertension (PH) is a serious medical condition that affects the blood vessels in the lungs. It is important not to confuse it with the more common form of high blood pressure (arterial hypertension). In pulmonary hypertension, the pressure inside the blood vessels of the lungs is increased. As a result, the right side of the heart, particularly the right ventricle, has to work harder to pump blood through the lungs. Over time, this extra strain can weaken the heart muscle, eventually leading to right heart failure if the condition is not treated.
In many patients, the exact cause of pulmonary hypertension cannot be identified. This type is known as idiopathic pulmonary arterial hypertension (IPAH), previously referred to as primary pulmonary hypertension. IPAH is a rare condition and is seen more often in women than in men. Although the cause is unknown, it is a well-recognised and carefully studied form of the disease.
When pulmonary hypertension develops as a result of another medical condition, it is called secondary pulmonary hypertension. There are several known causes. These include congenital heart disease, blood clots in the lungs (pulmonary embolism), left-sided heart disease such as heart failure or valve problems, and chronic lung diseases like chronic obstructive pulmonary disease (COPD) or pulmonary fibrosis. Other causes include long-term low oxygen levels in the blood, HIV infection, autoimmune diseases such as scleroderma and rheumatoid arthritis, obstructive sleep apnoea, and, in some cases, certain medications (including some older weight-loss drugs).
The most common symptom of pulmonary hypertension is shortness of breath, particularly during physical activity. Many patients also experience fatigue, dizziness, or even fainting episodes (blackouts). Swelling of the legs or ankles (oedema) can occur as the condition progresses, and some people may feel chest discomfort or pain, especially when exerting themselves. Because these symptoms can be mild at first and develop gradually, they are sometimes mistaken for other conditions.
Pulmonary hypertension is diagnosed through specialist assessment. This may include tests such as an echocardiogram (ultrasound of the heart), blood tests, imaging of the lungs, and sometimes a right heart catheterisation, which is the most accurate way to confirm the diagnosis. It is important that diagnosis and management take place in a specialist centre with experience in pulmonary hypertension.
Treatment depends on the underlying cause and the severity of the condition. In many cases, specific medications are used to help relax and widen the blood vessels in the lungs, improving blood flow and reducing strain on the heart. These include drugs such as Bosentan, Iloprost, and Sildenafil. Some patients may also require additional treatments such as oxygen therapy, diuretics to reduce fluid retention, or anticoagulants. In selected cases, surgical or interventional treatments may be considered, particularly when there is an identifiable underlying cause.
Pulmonary hypertension can sometimes be treated with surgery, particularly when it is caused by old blood clots in the lungs. For this reason, every patient with unexplained pulmonary hypertension should be assessed for possible blood clots. This condition is called chronic thromboembolic pulmonary hypertension (CTEPH). Current international guidelines recommend screening for CTEPH because it is one of the few potentially curable forms of pulmonary hypertension. CTEPH is usually investigated using a ventilation/perfusion (V/Q) scan, which is the most sensitive screening test, along with a CT pulmonary angiogram to look at the blood vessels in more detail.
If these tests suggest CTEPH, patients should be referred promptly to a specialist pulmonary hypertension centre, where a multidisciplinary team can assess whether surgery or other treatments are appropriate. The main surgical treatment for CTEPH is called a pulmonary endarterectomy (PEA). This is a highly specialised operation in which surgeons remove old, organised blood clots from the pulmonary arteries to restore normal blood flow to the lungs. During the procedure, the patient is connected to a heart-lung (extracorporeal circulation) machine, which temporarily takes over the work of the heart and lungs. This allows the surgeon to safely open the pulmonary arteries and carefully remove the obstructing material.However, not all patients are suitable for surgery, and in some cases, pulmonary hypertension may persist or recur even after a technically successful operation. These patients may require ongoing treatment with specialised medications used for pulmonary hypertension, along with regular follow-up in a specialist centre. After surgery, lifelong anticoagulation (blood-thinning treatment) with Warfarin is recommended to prevent new clots from forming, although the exact treatment plan is tailored to each patient based on current clinical guidance.
In the UK, assessment and surgical treatment for CTEPH are nationally centralised at Royal Papworth Hospital NHS Foundation Trust, ensuring patients are managed by highly experienced teams.
Pulmonary hypertension is a progressive condition, but early diagnosis and appropriate treatment can make a significant difference. With modern therapies and specialist care, many patients experience improvement in symptoms and quality of life. Anyone experiencing persistent or unexplained breathlessness, especially during activity, should seek medical advice for further evaluation.
