Hypoplastic left heart syndrome (HLHS) was a uniformly fatal diagnosis before 1983, when surgical treatment was first undertaken with the Norwood I operation as the first of 3-staged operations. Since then, operative survival rate of stage I has risen from 53% to over 90% in the current era, not only thanks to technical advances in surgery but also through prenatal diagnosis and imaging, enhanced cardiopulmonary bypass technology, better perioperative intensive care, and closer interstage monitoring. The improvements in patient outcomes achieved through rigorous multidisciplinary teamwork have come at a tremendous cost in manpower and resources, making HLHS still a challenge to all congenital heart programs, established or emerging. We review the various surgical steps to treat HLHS and their current expected outcomes, and put into perspective cost considerations compared to other more "simple" congenital heart defects.
Keywords: Norwood; congenital heart disease; hypoplastic left heart syndrome.