Clinical, functional and hemodynamic profile of patients with Eisenmenger syndrome in comparison with the group of idiopathic pulmonary hypertension, features of starting specific therapy choice

Aim. To conduct a comprehensive analysis of the clinical, functional, hemodynamic profile of patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) and Eisenmenger syndrome, as compared with the group of idiopathic pulmonary hypertension (IPAH) according to the Russian Registry, and also to study the features of drug therapy. Materials and methods. The study involved 20 patients with PAH-CHD and Eisenmenger syndrome and 20 patients with IPAH over 18 years old. All patients had been entered in the Russian registry (NCT03707561). A comparative analysis of clinical, functional, hemodynamic parameters was held. The diagnosis was established in accordance with the algorithm proposed in the Russian guidelines the diagnosis and treatment of PH (2016). Results. In this study, 20 patients with PAH-CHD (16 women and 4 men) and Eisenmenger syndrome and 20 patients with IPAH (18 women and 2 men) were analyzed. Patients were comparable in age and sex. The time from the onset of symptoms to the final diagnosis in the PAH-CHD group was significantly longer: on average, it took three years to establish the diagnosis, whereas in patients with IPAH, on average, after 10.5 months, the correct diagnosis was made (p=0,0006). In the group of patients with Eisenmenger syndrome, there was a significant decrease in SpO2 both at rest and under exertion and averaged 86.5±9.7% at rest and 70±12.3% after exercise (p=0,0002). Conclusions. Patients with Eisenmenger syndrome compared with the IPAH group are characterized by a longer duration of symptoms until the diagnosis, and lower oxygen saturation. When estimating the parameters of pressure in the pulmonary artery (sPAP, dPAP, mPAP), according to the data of right heart catheterization and echo, significantly higher values of pressure in the pulmonary artery and lower values of SpO2 were detected.

pulmonary arterial hypertension, congenital heart disease, Eisenmenger syndrome, idiopathic pulmonary hypertension

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