Abstract
The surgical treatment of a chronic thromboembolic pulmonary hypertension (CTEPH) by pulmonary endarterectomy (PEA) is a highly effective method of treatment. However, only 60% of patients undergo PEA due to anatomical types of impairment and their comorbidities.
Patients who are not suitable for surgical intervention and patients with residual pulmonary hypertension after PEA are candidates for a specific pharmacology treatment of CTEPH with vasodilators. The pharmacology treatment of CTEPH has recently included only a permanent anticoagulation treatment and a symptomatic therapy of the chronic right heart failure with diuretics.
The introduction of the specific treatment of CTEPH with vasodilator riociguat significantly improves patients' symptoms. The case report presents the effectiveness of that therapy and it also discusses potential risks associated with riociguat.