Main prognostic parameters in PAH are based on clinical symptoms (clinical signs of RV failure, progression of symptoms, syncope), World Health Organization (WHO) functional class, 6MWT, laboratory (NT-proBNP level), hemodynamics, and cardiopulmonary exercise testing, as well as different imaging modalities such as echocardiography or CMR [9]. This evidence concerns the gene NPPB and pulmonary arterial hypertension.