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.
- Transthoracic echocardiography estimates probability and right-heart impact; it does not confirm haemodynamics or define operability.
- Ventilation–perfusion scanning is a sensitive screening test for chronic thromboembolic obstruction. A routine CTPA reported as negative for acute PE may miss chronic distal disease.
- Right-heart catheterisation confirms the haemodynamic phenotype, while high-quality pulmonary vascular imaging maps disease for treatment decisions.
- Every confirmed patient should be assessed by a specialist CTEPH multidisciplinary team for pulmonary endarterectomy, regardless of age alone or initial local assumptions about operability.
- Pulmonary endarterectomy can be curative in operable disease; balloon pulmonary angioplasty and specialist drug therapy are options for selected inoperable, persistent or recurrent disease.
- Therapeutic anticoagulation is generally long term, but agent choice is individualised by the specialist team, antiphospholipid status, bleeding risk, pregnancy and comorbidity.
- Exact targeted-therapy eligibility is commissioning- and jurisdiction-dependent; NHS England updated its adult pulmonary-hypertension targeted-therapy policy in July 2026.
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.
New pleuritic pain, haemoptysis, tachycardia or acute hypoxaemia during follow-up may represent recurrent PE despite treatment and needs immediate reassessment of adherence, interaction, absorption and thrombosis drivers.
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.
- Refer abnormal perfusion or convincing clinical findings to a designated pulmonary-hypertension centre rather than repeating nonspecialist CT alone.
- Escalate urgently for right-heart decompensation, syncope or rapid deterioration.