Scope: The aim of the study was to provide updates of the actual approaches of children care with pulmonary hypertension (PH).

Methods: We present a review based on the research of literature, data from health-related bibliographic data-bases such as Hinari (WHO), Pubmed. Also it contains current recomandations from the Sixth World Symposium on Pulmonary Hypertension in Nice, 2018 and the latest Consensus 2019, approved by the European Association for Lung Vascular Diseases and other experts.

Results: The 6-th WSPH proposed to modify the definition for pulmonary hypertension as mPAP >20 mmHg (versus >25 mmHg) and to include the use of pulmonary vascular resistance as indexed to body surface area PVR ≥3 WU to identify pre-capillary PH. The updated clinical classification includes a separate designation for congenital/acquired cardiovascular conditions leading to post-capillary PH, developmental lung disorders, other pulmonary artery obstructions and complex congenital heart disease. Treatment strategies and new clinical end-points. The choice of the initial agent for PH treatment is largely based on the tests results of acute vasoreactivity and disease severity. In children with a positive acute vasoreactivity test response, we should initiate calcium channel blockers. In children with a negativ response, initiation of oral monotherapy is recommended. Treatment of choice is an endothelin receptor antagonist – bosentan, ambrisentan or phosphodiesterase type 5 (PDE5) inhibitor – sildenafil, tadalafil. Children who deteriorate on either endothelin receptor antagonists or PDE5 inhibitors, should benefit from consideration of early combination therapy with addition of inhaled prostacyclin, iloprost or treprostinil. In patients with severe PH, early consi-deration interventional palliative bridges is important.

Conclusions: Pulmonary hypertension is associated with a considerable risk of morbidity and mortality. Promoting current specific recommendations for children involves implementing appropriate management which foretells the possibility of a favorable prognosis.