Synopsis
Recognise chronic thromboembolic pulmonary hypertension after acute or occult pulmonary embolism, confirm it through a designated pulmonary-hypertension pathway, and prioritise potentially curative pulmonary endarterectomy.
- CTEPH is persistent organised thromboembolic obstruction with pulmonary vascular remodelling and pulmonary hypertension; it is not simply residual clot visible after an acute pulmonary embolism.
- Suspect it when breathlessness, exercise limitation, syncope or right-heart signs persist after pulmonary embolism, but remember that some patients have no documented acute embolic event.
- A normal or mildly abnormal chest radiograph and nonspecific ECG do not exclude significant disease.
Key red flags
Rest symptoms, syncope, hypotension, rising JVP, cool peripheries, hepatic congestion, renal dysfunction or escalating oxygen requirement indicate advanced disease and require urgent specialist assessment.
Investigation priorities
Identify right-heart strain, competing lung or cardiac disease and consequences of congestion.
Management branches
Breathlessness, syncope or exercise limitation fails to improve as expected after treated PE.
- Confirm anticoagulant adherence, dose appropriateness, interactions and whether a new acute PE or another cardiopulmonary diagnosis is present.
- Obtain ECG, chest radiograph, echocardiography and baseline physiology, then arrange V/Q scanning when CTEPH remains possible.
Key medicines
Long-term therapeutic anticoagulationUse an individually selected full-treatment regimen continuously, verified against the current BNF, renal function, interactions and specialist CTEPH plan.
RiociguatInitiated and titrated only by a designated pulmonary-hypertension centre under the current NHS commissioning policy and product information.