Pulmonary hypertension
Also called: PH, PAH
High pressure in the arteries of the lungs strains the right side of the heart. Breathlessness and fatigue worsen over time; untreated it leads to right heart failure.
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Causes
- Left heart disease (most common)
- Lung disease and low oxygen (COPD, fibrosis, sleep apnoea)
- Chronic blood clots (CTEPH)
- Pulmonary arterial hypertension: idiopathic, inherited, drugs, connective tissue disease, HIV, congenital heart disease
Risk factors
- Heart and lung disease
- Previous pulmonary embolism
- Scleroderma
- Family history
Symptoms
- Breathlessness on exertion
- Fatigue
- Chest pain and fainting on exertion
- Ankle swelling
Diagnosis — tests and examinations
- Echocardiography
- Right heart catheterisation (confirms, mean pressure >20 mmHg)
- V/Q scan to exclude chronic clots
- Lung function and CT
Treatment
Medicines and medical treatment
- Treat the underlying heart or lung disease
- For PAH: endothelin receptor antagonists, PDE5 inhibitors, prostacyclins, sotatercept
- Anticoagulation for CTEPH
- Diuretics and oxygen
Operations and procedures
- Pulmonary endarterectomy for CTEPH
- Balloon pulmonary angioplasty
- Lung transplantation
Self-care, home remedies and lifestyle
- Gentle supervised exercise
- Avoid pregnancy in PAH (high risk)
- Vaccinations
Possible complications
- Right heart failure
- Arrhythmias
- Sudden death
Prevention
- Treat heart and lung disease; prompt treatment of pulmonary embolism
Outlook
Varies by cause; specialist treatment has substantially improved survival in PAH.
When to see a doctor
See a doctor for unexplained breathlessness or fainting on exertion.
Sources and further reading
These sources cover this condition. They have not been checked against every statement on this page.
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