Comparative clinical characteristics of patients with various forms of pulmonary arterial hypertension associated with congenital heart disease, according to the clinical classification

Objective: to conduct a comprehensive analysis of the clinical, functional, hemodynamic profile of patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) according to the Russian Registry. Materials and methods: The study involved 30 patients with Eisenmenger syndrome, 25 patients with PAH associated with prevalent systemic-to-pulmonary shunts and 26 patients with PAH after defect correction. All patients had been entered in the Russian registry (NCT03707561). A comparative analysis of clinical, functional, hemodynamic parameters was held. The diagnosis was established by the algorithm proposed in the Russian guidelines for the diagnosis and treatment of PH (2016).Results: The patients of three subgroups of PAH-CHD were comparable in age and sex. The time from the onset of symptoms to the final diagnosis in the first and second PAH-CHD subgroups was significantly longer: on average, it took three years to establish the diagnosis, whereas in patients with PAH after defect correction, on average, after 9 months the correct diagnosis was made (p=0,0006)...