Congenital heart defects are the most common type of birth defects, and with recent advances leading to improved survival, there are now over two million infants, children, and adults with congenital heart disease (CHD) in the United States alone (1-3). Many patients suffer from primary congenital valvular lesions or prosthetic/conduit valvular dysfunction. Given the substantial variability within each phenotype of CHD, this review will provide a basic background in embryology to allow the reader to create an adaptable construct in their approach to these patients (Table 1).As the prevalence of CHD grows, it is imperative that providers in all practice settings recognize pathology promptly, coordinate appropriate surveillance, and collaborate with CHD-specialists for optimal treatment. As such, this review provides details on the diagnosis, natural history, and contemporary therapeutic approaches to valvular heart disease in CHD. The information used to write this review was collected via computerized literature searches of electronic databases including U.S. National Library of Medicine (MEDLINE), ScienceDirect, and Google Scholar as well as hand searches of the references of the retrieved literature and discussions with experts in