Atypical Circular Shunt and Diffuse Emphysema in a Fetus with Double-Outlet Right Ventricle and Absent Pulmonary Valve.

• APVS is usually associated with agenesis of the ductus arteriosus. • APVS is frequently accompanied by dilation of the main and branch pulmonary arteries, often leading to tracheal and bronchial compression with varying degrees of respiratory distress. • A rare variation of APVS with circular shunt physiology is reported, expanding the clinical spectrum of the disease.