Abstract
The article has reported the clinical case of successful radical correction of the congenital absent pulmonary valve syndrome in a patient with
agenesia of the right pulmonary artery and right-sided aortic arch. According to our data the presented combination of congenital anomalies in
the cardiovascular system is very rare and has been described for the first time.
The congenital absent pulmonary valve syndrome has been associated with stenosis and hypoplasia of the pulmonary valve annulus with poststenotic
pulmonary artery dilatation. With large dilatation associated with compression of the trachea and bronchi the clinical symptoms in this
category of patients can include those of tracheoesophageal compression.
Parameters of structures of the heart and the single left pulmonary artery together with absence of respiratory distress and other complications
have allowed to perform the initial correction of intracardiac malformations.
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