[Pulmonary arterial hypertension: modern diagnostics and therapy--Part 1]

Pulmonary arterial hypertension (PAH) belongs to the group of rare diseases and the morbidity rate is 15 to 50 people per million per year. Before the era of specific treatment of PAH, the prognosis was poor. The average life expectancy of the patients was 2.8 years. However, in the last years there has been a breakthrough in treating the patients with PAH. The introduction of this specific treatment has prolonged the life and improved the quality of it within the group of the patients with PAH. In Poland, since 2008 the therapy has been organized by the Pulmonary Hypertension Therapeutic Program. PAH is a recognition done by excluding more probable causes of pulmonary hypertension (PH) such as: PH due to left heart disease and lung disease, chronic thromboembolic pulmonary hypertension (CTEPH) and PH with multifactorial mechanisms. The clinical symptoms of pulmonary hypertension are non-specific, they develop for a several months and they are mainly caused by progressive right ventricular failure. The base of PAH recognition is echocardiography, which indirectly estimates the pulmonary artery systolic pressure. However, the golden standard of PAH diagnostics is right heart catheterization (RHC) with measurements of pulmonary arterial pressure (PAP), right atrial pressure (RAP), right ventricular pressure and pulmonary wedge pressure (PWP). The early PAH recognition and the correct classification of patients to the treatment organized by the Pulmonary Hypertension Therapeutic Program give them a chance for longer and more comfortable life.