Prevalence of Pulmonary Hypertension in the Pediatric Population of the Russian Federation in 2021–2023: First Results from the National Registry

Background. The epidemiology of pulmonary hypertension (PH) in children in the Russian Federation has not been studied. Currently available data is limited to several foreign registries, mostly limited to the study of PH Group I cases. Objective. The aim of the study is to analyze the prevalence of PH in the pediatric population of the Russian Federation. Methods. Data on PH cases were collected from chief freelance pediatric cardiologists and pediatric cardiology specialists in 85 territorial entities of the country. Requests for data submission were sent January 2021, 2022, and 2023. The study included data from patients aged from 3 months to 17 years with established diagnosis of PH and available catheterization and/or echocardiography results. The PH diagnosis was verified based on mean pulmonary artery pressure (mPAP) values (> 20 mmHg for biventricular circulation and > 15 mmHg in univentricular hemodynamics) or, in their absence, systolic pulmonary artery pressure values (> 36 mmHg). PH clinical groups were created according to the diagnosis in medical records. Publicly available Russian Federal State Statistics Service data on pediatric population (total and by territorial entities) as of January 1, 2022 and 2023 were used to evaluate PH prevalence. Results . The study included data from549 patients with PH from 60 territorial entities of Russia from February 2021 to February 2023. Mean age was 10.0 (4.8) years, with 56% being female. The diagnosis was verified via cardiac catheterization in 71.5% of patients. The overall prevalence of PH was 22.5 cases (95% CI 18.3–27.4) per 1 million pediatric population; PH Group I (pulmonary arterial hypertension; PAH) accounted for 11.8 cases, including idiopathic PAH — 4.18 and PAH associated with congenital heart disease (CHD) — 7.16 per 1 million pediatric population. CHD was associated with 66.4% of all PH cases (Groups I and V). The highest prevalence (7.8 per 1 million pediatric population) was observed for PH cases associated with complex CHD. Conclusion . PH prevalence, its clinical groups and subgroups among pediatric population of Russian Federation is presented. The obtained data is crucial for evaluation of population's need for specialized medical care for this complex group of patients at both regional and federal levels.