Synopsis
Recognise pulmonary hypertension early, classify its cause correctly and refer suspected PAH or CTEPH to nationally commissioned specialist care.
- Pulmonary hypertension is haemodynamically defined by mean pulmonary artery pressure greater than 20 mmHg at rest on right-heart catheterisation.
- Pre-capillary PH also requires pulmonary artery wedge pressure 15 mmHg or less and pulmonary vascular resistance greater than 2 Wood units.
- Echo estimates probability; it does not confirm PH or distinguish all five clinical groups.
Key red flags
Exertional syncope, hypotension, WHO functional class IV symptoms or rapidly worsening right-heart failure.
Investigation priorities
Look for RV strain, alternative disease, anaemia, renal/liver dysfunction, thyroid disease and treatment constraints.
Management branches
Unexplained dyspnoea, syncope, RV signs or a condition associated with PAH/CTEPH.
- Assess acuity, oxygen saturation, ECG, chest radiograph, FBC, renal/liver/thyroid tests and BNP/NT-proBNP.
- Obtain expert echocardiography to grade PH probability and examine left heart, RV and shunts.
Key medicines
Sildenafil20 mg orally three times daily for PAH.
Bosentan62.5 mg orally twice daily for 4 weeks, then 125 mg twice daily if tolerated.