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Unilateral Absence of Left Pulmonary Artery with Absent Pulmonary Valve Syndrome: Surgical Perspective on Primary Repair

Sachin Mahajan, Sudhansoo Khanna, Ruchit Patel, Vikram Halder, Vidur Bansal

Tetralogy of Fallot (TOF) with absent pulmonary valve and unilateral absence of pulmonary artery is an extremely rare congenital cardiac anomaly. Historically, the outcome of surgery in this subset has been very dismal. We believe valveless reconstruction for Right Ventricular Outflow Tract (RVOT) as a part of primary repair should never be opted for in this variant. Only a fully competent and durable valve in RVOT can provide meaningful early and time related survival in this variant. We report our experience with a bi-leaflet pulmonary valve constructed from 0.1 mm PTFE membrane in this substrate.