The prevalence of congenital heart defects (CHDs) in adults has increased during the last few decades such that now there are more adults with CHD than children. Advances in the diagnosis and management of CHD in children has resulted in this phenomenon. This review indicated that transcatheter methods are successful in relieving obstruction associated with coarctation of the aorta, pulmonary stenosis and aortic stenosis. With regard to cyanotic CHD, the majority of patients presenting in adulthood are likely to be those that have had corrective or palliative surgery during infancy and childhood, although on rare occasion uncorrected defects may present for the first time in adulthood. Pathophysiologic effects of right to left shunt associated with cyanotic CHD are reviewed. Brief description of the anatomy of the most common cyanotic CHDs, namely, tetralogy of Fallot, transposition of the great arteries, tricuspid atresia, total anomalous pulmonary venous connection, and truncus arteriosus was presented followed by a discussion of management strategies of the residual defects. Adults who did not have surgical correction in childhood should undergo surgical correction, but they may have a higher risk than that seen in children