Chronische trombo-embolische pulmonale hypertensie

• Chronic thromboembolic pulmonary hypertension (CTEPH), characterised by pulmonary hypertension and persistent perfusion defects despite adequate anticoagulation, causes significant morbidity and mortality.
• Persistent dyspnoea after acute pulmonary embolism is frequent and an indication for additional diagnostics. Only a minority of these patients develop CTEPH.
• Echocardiography and perfusion scintigraphy are the cornerstone of diagnostics when suspecting CTEPH. Right-heart catheterisation and pulmonary angiography should confirm the diagnosis.
• The diagnostic phase is preferably carried out in an expert centre in order to optimise the diagnosis and choose the optimal treatment for each individual patient.
• Treatment of patients with CTEPH is a multidisciplinary team effort.
• Pulmonary endarterectomy is the only potentially curative treatment; perioperative mortality is less than 5% in experienced centres. Inoperable patients can be treated with medication that specifically targets pulmonary arterial hypertension, but a survival benefit has not yet been shown for this medication.
• Balloon pulmonary angioplasty has recently become available in the Netherlands as a treatment option, but the exact role of this new technique in the treatment of patients with CTEPH still needs to be investigated.

Conflict of interest and financial support: ICMJE forms provided by the authors are available online along with the full text of this article.