Tetralogy of Fallot

Abstract

TOF is seen in patients with DiGeorge syndrome and may be present in combination with AVC defect in children with trisomy 21. Harsh systolic ejection murmur is detected in the first week of life. Hypercyanotic spells are life threatening and typically not seen in the first few months of life. This may be induced by dehydration. Surgical repair is planned at about 4–6 months of age. Severe restriction to pulmonary blood flow due to pulmonary stenosis or atresia may initially require a systemic to pulmonary arterial shunt. Free (severe) pulmonary regurgitation resulting from surgical enlargement of pulmonary valve annulus eventually causes right ventricular dilation and fibrosis which ultimately may result in ventricular arrhythmias. Severe pulmonary regurgitation after TOF repair should be corrected by surgery or interventional cardiac catheterization (currently experimental). Homograft valves (and other biological material) are used for this type of repair.