Congenital heart diseases are an important cause of mortality and morbidity in pedatric patients. Fallot Type Double outlet right ventricle is a rare heterogenous congenital heart disease characterized by a complex group of lesions similar with Tetralogy of Fallot. Surgical approach depends on the anatomy of associated lesions, therefore in order to perform a successful correction, a careful review of the anatomy and a detailed diagnostic is needed due to complexity of intracardiac anomalies. The main diagnostic tool used nowadays for the diagnosis of double outlet right ventricle is echocardiography exam, a non-invasive examination that can provide information about anatomical features, but it will not allow the full description of the coronary anatomy. Therefore, preoperative assessment should include, extensive imaging assessment (echocardiography exam, angio-CT exam) for a correct management of surgical strategy prior to surgical correction.
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