Complete atrioventricular (AV) canal defect with double outlet right ventricle (DORV) and severe pulmonary stenosis (PS) is a rare complex congenital heart disease, requiring surgical correction usually within 6 months… Click to show full abstract
Complete atrioventricular (AV) canal defect with double outlet right ventricle (DORV) and severe pulmonary stenosis (PS) is a rare complex congenital heart disease, requiring surgical correction usually within 6 months of life. Patients with the unrepaired lesion can develop elevated pulmonary venous pressures, which over the time become fixed and they develop reversal of shunt and cyanosis (Eisenmenger syndrome).[1] The lifespan of the patient depends on the balance between the pulmonary and systemic circulations.[2]
               
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