Abstract
Double-outlet right ventricle is a complex congenital heart disease characterized by an abnormal of ventriculo-arterial connection when aorta and pulmonary trunk are completely or predominantly derived from the right ventricle. The rate of the malformation among all congenital heart abnormalities is 0.7–2.7%. The condition of these patients is regarded as severe due to signs of chronic arterial hypoxemia or heart failure, depending on the form of the defect. In general, the median survival rate does not exceed 5 years and only 17% of patients live to 15 years without surgical treatment. These clinical guidelines represent a current undestanding of etiopathogenesis, the clinical picture, diagnosis, conservative and surgical treatment of double outlet right ventricle.
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