We present a rare case of a two-week-old infant with tetralogy of Fallot , absent pulmonary valve syndrome , right aortic arch, and disconnected left pulmonary artery (LPA) whose origin was from ductal ligament adjacent to the left subclavian artery. One-stage surgical correction, including closure of ventricular septal defect (VSD), LPA reconstruction and reconnection to the pulmonary trunk, reduction in size of the right pulmonary artery (RPA), and right ventricular outflow tract reconstruction with valved conduit, was successfully performed with good clinical mid-term outcome.
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