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Various combinations of congenital heart defects are included in complex congenital heart disease. Various pathologies of cardiac structures have varied embryological dysmorphology. Lesions described are single ventricle pathologies, hypoplastic left heart syndrome, tricuspid atresia, pulmonary atresia and intact ventricular septum, and crisscross atrioventricular connections. Irrespective of underlying structural abnormalities, most of these lesions will need a unified single ventricle pathway of palliative surgeries for continued survival. A few complex lesions are amenable to corrective surgery. Approach to diagnosis, echocardiography of different lesions, a unified approach toward treatment is discussed.