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The tetralogy of Fallot (TOF) with an absent pulmonary valve is a rare congenital cardiac malformation. We recorded the fetal echocardiographic findings for a fetus with the TOF with absent pulmonary valve and review the literature on this condition. The infant died 5 days after birth and the autopsy showed no infundibular stenosis. The degree of infundibular stenosis may be correlated with the prognosis of infants with this disease.
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