Abstract
A syndrome of hypoplasia of the right ventricle is a very rare congenital anomaly, in which inlet or apical trabecular
part of the right ventricle is absent or underdeveloped. The anomaly of structure and hemodynamics takes
intermediate place among heart diseases with a single ventricle and heart diseases with biventricular circulation.
Hypoplasia of the right ventricle prevents the formation of a normal biventricular circulation. The attempt to divide
ventricles of different sizes with a surgical method leads to inability of the right ventricle to provide pulmonary
blood flow independently. The main failures for the correction of a congenital heart disease are connected with partial
diagnostics and inadequate surgical approach. If you define quantitative variables of RV, it will help to choose
the right approach. It is stated that hypoplasia influences on hemodynamics when right ventricular diastolic volume
is less than 80% from the standard. Significant presentations of the degree of hypoplasia can be obtained with
echocardiography and angioventriculography.
Radical correction with bidirectional cavopulmonary anastomosis allows preventing the development of acute
right ventricular dysfunction in postoperative period in patients with complex congenital heart diseases and right
ventricular hypoplasia. Besides, correction of congenital heart disease is possible in children who had insult and
have severe organic changes in brain substance after previous meticulous examination with computerized tomography
and having a qualified opinion of a neurologist.
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