Presented, is the case of a newborn with left-sided lung agenesis associated with total anomalous pulmonary venous return, atrial septal defect, polysplenia and hypospadias was described. Throughout the entire hospitalization period the newborn was in poor general condition, and dependent on mechanical ventilation with high oxygen concentrations. On day 20, the newborn died. According to the available literature, this case is unique in regard to the type and number of malformations.
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