Abstract
The prevalence of congenital heart disease has accounted for nearly one-third of all significant congenital anomalies worldwide. The first report about an association between cardiac anomalies and Down Syndrome was in (1876). Ten years after discovering of Down Syndrome and the credit of association between congenital cardiac anomalies and mongolism was suggested in (1894) by Garrod. There many studies performed to identify a correlation between genotype and phenotype in Down Syndrome, little is known about cardiovascular phenotype in Down Syndrome. Congenital heart disease is considered one of the highest causes of mortality and morbidity in Down Syndrome compared to patients with the same lesion of non-down. There is a big debate about surgical management and considered them as risk factors of surgery with precaution and recent technology, Down Syndrome considered as a normal patient in prognosis. This chapter aimed to shed the light on congenital heart disease in Down Syndrome and current knowledge in specific mutations associated with them and how the effect of innovative technology and management to treat them end at the same outcome and sometimes better based on recent research and Scoring System.