10-year female child was presented with complex congenital heart defect—atrial situs inversus,
levocardia, discordant atrioventricular connections, double outlet right ventricle, ventricular
septal defect, pulmonic stenosis and straddling of atrio-ventricular valves along with bilateral superior
vena cava and large patent ductus arteriosus. We describe here the strategy adopted to
perform single stage fenestrated extra cardiac conduit total cavo-pulmonary connections in this
patient especially concerning cardiac malposition of atrial situs inversus and levocardia.
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