Treating underlying conditions
If pulmonary hypertension is caused by another condition, such as a heart or lung problem, treatments will focus on the underlying condition.
You may also be offered an operation known as a pulmonary endarterectomy.
Specialist centres for pulmonary arterial hypertension
If you have pulmonary arterial hypertension (PAH), you'll be referred to a centre that specialises in treating this form of the condition. There are seven centres in England and one in Scotland.
Treatments for pulmonary arterial hypertension
There are many treatments for pulmonary arterial hypertension (PAH). Which treatment or combination of treatments you'll be offered will depend on a number of factors, including what's causing PAH and the severity of your symptoms.
- anticoagulant medicines – such as warfarin to help prevent blood clots
- diuretics (water tablets) – to remove excess fluid from the body caused by heart failure
- oxygen treatment – this involves inhaling air that contains a higher concentration of oxygen than normal
- digoxin – derived from the foxglove plant, digoxin can improve your symptoms by strengthening your heart muscle contractions and slowing down your heart rate
There are also a number of specialist treatments for PAH that help relax the arteries in the lungs and reduce the blood pressure in the lungs.
These medicines slow the progression of PAH, and may even reverse some of the damage to the heart and lungs.
- endothelin receptor antagonists – such as bosentan, ambrisentan and macitentan
- phosphodiesterase 5 inhibitors – sildenafil and tadalafil
- prostaglandins – epoprostenol, iloprost and treprostinil
- soluble guanylate cyclase stimulators – such as riociguat
- calcium channel blockers – nifedipine, diltiazem, nicardipine and amlodipine
You can find detailed information about these treatments for pulmonary hypertension on the Pulmonary Hypertension Association UK website.
Surgery and procedures for pulmonary hypertension
Some people with pulmonary hypertension may need surgery. The three types of surgery currently used are:
- pulmonary endarterectomy – an operation to remove old blood clots from the pulmonary arteries in the lungs in people with chronic thromboembolic pulmonary hypertension
- balloon pulmonary angioplasty – a new procedure where a tiny balloon is guided into the arteries and inflated for a few seconds to push the blockage aside and restore blood flow to the lung; it may be considered if pulmonary endarterectomy isn't suitable, and has been shown to lower blood pressure in the lung arteries, improve breathing, and increase the ability to exercise
- atrial septostomy – a small hole is made in the wall between the left and right atria of the heart using a cardiac catheter, a thin, flexible tube inserted into the heart's chambers or blood vessels; it reduces the pressure in the right side of the heart, so the heart can pump more efficiently and the blood flow to the lungs can be improved
- transplant – in severe cases, a lung transplant or a heart-lung transplant may be needed; this type of surgery is rarely used because effective medication is available
The National Institute for Health and Care Excellence (NICE) has guidance on balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension.