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Congenital long QT syndrome (LQTS) is a rare cardiac condition characterized by abnormality of either sodium or potassium ion channels resulting in prolongation of QT interval and thereby predisposing to life-threatening arrhythmia. Once the syndrome is diagnosed, measures should be taken to avoid sudden cardiac death. We present a rare case of LQTS associated with patent ductus arteriosus in a child, and a unique approach was used in managing both conditions.Total anomalous pulmonary venous drainage (TAPVD) encompasses a wide spectrum