Contemporary Strategy of Pulmonary Hypertension Management in Pediatrics

Abstract

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.

Pulmonary Hypertension in Children - New Insights of Diagnosis and Management

“…There is a broad consensus that a diagnostic cardiac catheterization with acute vasoreactivity testing be undertaken at least once in any patient with signifi cant pulmonary hypertension, mainly if specifi c pulmonary hypertensive therapy is recommended. In general, it is welcomed to repeat cardiac catheterization 6-12 months after initiating targeted PHVD therapy 3,7 . Endothelin-1 (ET-1) represents the key mediator in the pathogenesis of IPAH, with high concentrations in the plasma.…”

Pulmonary hypertension associated with congenital portosystemic shunts as a new form of portopulmonary hypertension in children and adults

“…Пациентам с верифицированной прекапиллярной ЛГ на фоне ВПКШ следует рассмотреть как можно более раннее назначение ЛАГ-специфической терапии, объем которой (моно-, двой ная или тройная) определяется на основании результатов стратификации риска неблагоприятных событий, включая летальность [1,2]. В случае, если признаки легочной васкулопатии выявлены, любому хирургическому лечению ВПКШ должно предшествовать таргетная ЛАГ-специфическая терапия из-за риска периоперационных осложнений [18][19][20]27]. Показания к трансплантации печени при ППГ включают печеночную энцефалопатию, неподдающуюся медикаментозному лечению, опухоли печени, такие как гепатобластома, фокальная узловая гиперплазия и сопутствующие пороки развития, такие как атрезия желчевыводящих путей.…”