Abstract
This Russian guidelines were made on the base of published international guidelines as well as on the basis of the experience of treatment
for more than 25 000 children with congenital heart diseases and pulmonary hypertension at A.N. Bakoulev Scientific Center
for Cardiovascular Surgery.
Were clarify the definition and classification of pulmonary hypertensive vascular disease, detailed algorithms its diagnostic, surgical
and medical treatment with available drugs in the Russian Federation.
Particular attention is paid to issues not covered in the current international recommendations: methodology of vasoreactivity testing
assessing, assessment of operability in children with congenital heart diseases, pulmonary hypertension in patients with univentricular
haemodynamic. It demonstrates this role of age as a criterion of the severity of pulmonary vascular disease.
Separately examined the role of palliative surgery for congenital heart defects with pulmonary vascular disease.
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