Tetralogy of Fallot with Absent Pulmonary Valve

Tetralogy of Fallot (TOFTetralogy of Fallot (TOF)) with absent pulmonary valveAbsent Pulmonary Valve is a relatively rare disease, characterized by absence of the pulmonary valve that results in a stenotic and regurgitant RVOT, leading to very enlarged and pulsatile proximal pulmonary arteries. TOFTetralogy of Fallot (TOF) with absent pulmonary valveAbsent Pulmonary Valve accounts for 3–6% of all TOFTetralogy of Fallot (TOF). The clinical presentation varies depending on extent of pulmonary artery enlargementPulmonary Artery Enlargement, airway compressionAirway Compression and respiratory compromise. Severely compromised neonates require immediate postnatal medical management to ensure adequate ventilation and oxygenation. If possible, symptomatic patients should be stabilized to promote somatic growth. Surgical repair focuses on reduction of the enlarged branch pulmonary arteries with or without implantation of a valved conduit. Early surgical mortality is low, but long-term survivalSurvival can be significantly limited, and the reintervention rate is high. Reinterventions are primarily performed for recurrent airway disease, pulmonary valve, and pulmonary artery complications.