Contemporary strategy of pulmonary hypertension management in pediatrics

Pulmonary hypertension (PH) in pediatrics is a polygenic multifactorial condition with extremely adverse prognosis. Selection of optimal management is a severe task. In absence of treatment the mean life duration in children is not higher one year. Last two decades, revolution in approaches to treatment improved the survival of this patients group. Recently, pediatricians and pediatric cardiologists have three drugs groups that act on the main pathogenetic chains of PH: endothelin pathway, nitric oxide pathway and prostacyclin pathway. At the moment, approaches to pediatric PH are based on the data obtained in the trials on adult patients. However, not long ago there were first randomized trials on children performed. The group of authors of current article presents a modern view on the problem of PH in children, and expert recommendations on children management. Class of recommendations and evidence level were set by the data obtained in pediatric population or on adult population with at least 10%of children included. To the strategy, developed by the Russian clinicians, laid the analysis of experience of the pathology treatment in Russian Federation, as the current practics and clinical guidelines on pediatric PH in Europe, and the recent trials published. © 2018 Vserossiiskoe Obshchestvo Kardiologov. All rights reserved.

Miklashevich I.M.1 , Shkolnikova M.A.1 , Gorbachevsky S.V.2 , Shmalts A.A.2 , Groznova O.S.1 , Sadykova D.I.3 , Yakovleva L.V.4 , Degtyarev D.N.5 , Burov A.A.5 , Ovsyannikov D.Yu. 6 , Volkov A.V. 7
Vserossiiskoe Obshchestvo Kardiologov
Номер выпуска
  • 1 Veltishchev Scientific-Research Clinical Institute of Pediatrics, Pirogov RNRMU, Moscow, Russian Federation
  • 2 Bakulev Scientific Center of Cardiosurgery, Moscow, Russian Federation
  • 3 Kazanskaya State Medical Academy, Kazan, Russian Federation
  • 4 Bashkirskiy State Medical University, Ufa, Russian Federation
  • 5 Kulakov Scientific Center of Obstetrics, Gynecology and Perinatology, Moscow, Russian Federation
  • 6 RUDN University, Moscow, Russian Federation
  • 7 Nasonova Scientific-Research Center of Rheumatology, Moscow, Russian Federation
Ключевые слова
Atrial septostomy (atrioseptostomy); Children; Heart failure; Medications; Potts anastomosis; Pulmonary hypertension; Randomized controlled study
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