[Chronic Thromboembolic Pulmonary Hypertension]

Chronic thromboembolic pulmonary hypertension (CTEPH) occurs as a late complication of acute pulmonary embolism (PE). Scar-like narrowing of the pulmonary circulation resulting from insufficient thrombolysis and variable microvasculopathy increase the pulmonary vascular resistance. Early detection is particularly important because effective, potentially curative treatment options exist. Diagnosis and treatment planning require high-quality imaging combined with hemodynamic evaluation via right heart catheterization, which should be performed at specialized centers with multidisciplinary teams. If possible, patients should be treated with surgical pulmonary endarterectomy. If this is not possible, repeated balloon angioplasties are used for suitable target lesions, and both mechanical procedures are supplemented with medical therapy if the distal component is concomitant. Multimodal treatment concepts can achieve excellent results with a 3-year survival rate of over 90 %.