Congenital heart defects (CHD) occur in approximately 0.8% of live births and are a leading cause of death in children with congenital malformations. The etiology is primarily unknown and likely involves a mix of genetic and environmental factors, requiring careful evaluation through history, physical examination, and diagnostic imaging. Classification of CHD includes structural defects, cardiomyopathies, and arrhythmias, with final diagnosis confirmed via echocardiography, CT, MRI, or cardiac catheterization.