Absent pulmonary valve syndrome (APVS) is a rare congenital cardiac anomaly, commonly associated with tetralogy of Fallot (TOF). Non-TOF variant, APVS with intact ventricular septum (IVS) and patent ductus arteriosus (PDA) are infrequently reported. While airway compression is a well-known complication, hemodynamic compromise with circular shunting is extremely rare. A full-term neonate with prenatally diagnosed APVS and IVS presented with severe pulmonary stenosis, pulmonary regurgitation (PR), tricuspid regurgitation (TR), and a large PDA. On the 3 rd day of life, the infant developed respiratory distress and systemic hypoperfusion. Echocardiography revealed a circular shunt comprising severe PR and TR, right-to-left shunting at the atrial level, and left-to-right ductal flow. Emergent PDA ligation resulted in rapid hemodynamic recovery. The infant remained well at the 8-month follow-up. This appears to be the first reported case of circular shunting in APVS and intact IVS, emphasizing the need for vigilance and timely surgical intervention.
Prakash et al. (Thu,) studied this question.