Pavel Jansa, Iveta Šimková, Jaroslav Lindner, Michael Aschermann, Aleš Linhart
Chronic thromboembolic pulmonary hypertension (CTEPH) is one of the leading causes of severe pulmonary hypertension. The first treatment option for symptomatic patients with CTEPH is pulmonary endarterectomy. The success of surgery is dependent on patient suitability. Between 10 % and 30 % patients with CTEPH may not be eligible for surgery due to distal disease or serious comorbidities. There is some evidence that drugs approved for the treatment of PAH (prostanoids, endothelin receptor antagonists, phosphodiesterase-5 inhibitors) can provide benefits in these patients. First-ever double-blind study in inoperable patients with CTEPH was performed with bosentan. Lung transplantation is an option in cases where pulmonary endarterectomy or medical therapy failed.