While congenital heart defects are typically diagnosed in early childhood, depending on the severity of the lesion, they may not be recognized until later childhood or in adulthood. Left heart pressure loading lesions, including mitral valve stenosis, subaortic stenosis, valvular aortic stenosis, supravalvular aortic stenosis, and coarctation of the aorta, may be found as an isolated lesion or as a constellation together known as Shone syndrome. This review will highlight the literature published regarding the diagnosis, intervention, and long-term management of left heart pressure loading lesions.