Synopsis
Recognise pulmonary arterial hypertension as a specialist haemodynamic diagnosis, distinguish it from pulmonary hypertension caused by left-heart, lung or thromboembolic disease, and escalate risk-directed care safely.
- Pulmonary hypertension is a haemodynamic state with several causes; pulmonary arterial hypertension is the pre-capillary group after chronic thromboembolism, significant lung disease and left-heart disease have been excluded.
- PAH may be idiopathic, heritable, drug-associated or linked to connective-tissue disease, congenital heart disease, portal hypertension or HIV.
- Early symptoms are nonspecific: exertional breathlessness, fatigue, chest pressure and reduced exercise tolerance. Exertional presyncope or syncope is a high-risk feature.
Key red flags
Presyncope or syncope, rapid symptom progression, rest symptoms, hypotension, cool peripheries, rising natriuretic peptide, severe right-ventricular dysfunction or pericardial effusion indicates urgent specialist review.
Investigation priorities
Support pulmonary-hypertension suspicion, identify right-heart strain and provide prognostic baseline.
Management branches
Symptoms, high-risk condition or examination suggests pulmonary vascular disease.
- Assess urgency, oxygenation, ECG, chest radiograph, natriuretic peptide and routine causes of breathlessness including anaemia and thyroid disease.
- Obtain echocardiography and targeted lung assessment; do not diagnose PAH from symptoms or an echo estimate alone.
Key medicines
PDE5 inhibitor such as sildenafilInitiated at a designated pulmonary-hypertension centre using the current commissioned regimen and BNF, with dose review for interactions and organ dysfunction.
Endothelin-receptor antagonistSpecialist selection and dosing follow the current NHS policy, product monograph and centre protocol; combinations are risk- and eligibility-dependent.