Pulmonary Hypertension in Pediatric Practice

Abstract

Aim. The study was designed to review the Russian and foreign literature of recent years on modern methods of diagnosis and management of children with pulmonary hypertension.Materials and methods. The analysis of Russian and foreign literature was conducted in RSCI, Scopus, Web of Science, Pubmed databases.Results. Pulmonary hypertension (LH) is a rare, severe and progressive pathology with an unfavorable prognosis. For the first time, the clinical case of LH was described in the early XXth century, and only in recent decades, there were new drugs that could improve patients’ survival. The problem of LH is relevant in the clinical practice of a pediatrician. This pathology is often a complication of many cardiovascular and bronchopulmonary diseases in children. More than half of congenital heart defects in children are accompanied by LH. Bronchopulmonary dysplasia and interstitial lung lesions are also the leading causes of LH in the pediatric population. The evolution of drug therapy is associated with new ideas about the pathogenesis of the disease. If previously LH was associated with vascular tone disorders, recently, the pathogenesis is considered as a vascular remodeling, accompanied by a pathomorphological cascade of cell proliferation, hypertrophy, apoptosis, production and degradation of the intercellular matrix. However, despite the obvious breakthrough in the treatment of LH, the disease remains incurable and life-threatening.Conclusion.The problem of perfect diagnosis and effective treatment of LH is not completely solved nowadays. However, the progress made in pathogenetic therapy in the last decade allows us to hope for the emergence of new drugs that can significantly improve the prognosis and prolong the life of the patient.