Chronic thromboembolic pulmonary hypertension:Recognition, diagnosis and treatment

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.