Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare disease, but an important late sequela after acute pulmonary embolism. Therefore, follow-up after at least three months of sufficient anticoagulation is recommended. Patients with suspected CTEPH should be referred to specialized CTEPH centers for further evaluation and treatment.Three treatment modalities are available: pulmonary endarterectomy (PEA), balloon pulmonary angioplasty (BPA) and pulmonary hypertension-targeted drugs. The indication for surgery depends mainly on the localization of the pulmonary arterial obstructions. Severe comorbidities as well as advanced age need individual evaluation, but do not present strict exclusion criteria. Multimodal treatments are common practice in inoperable CTEPH. However, treatment decision making in an experienced multidisciplinary team is mandatory.
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