Current Outcomes and Considerations in Hypoplastic Left Heart Syndrome

Hypoplastic Left Heart Syndrome (HLHS) is a devastating congenital cardiac defect affecting approximately one thousand newborns annually in the United States. Without surgery, this condition is universally fatal. Currently, there are three management options for children born with HLHS: the Norwood palliative surgical procedure, neonatal cardiac transplantation, and comfort care without surgery. In the following review, I will first briefly describe the anatomy and embryology of HLHS. I will then describe the three management options, including published survival statistics, neurodevelopmental outcomes, and other considerations for infants receiving each of the treatment options. I will also describe new developments that may improve outcomes in the near future. Published data of parental choices in the care of infants born with HLHS, as well as published data on physician attitudes towards the three treatment options will be presented. Finally, I will offer guidelines for physician-parent discussions drawing from the informed consent literature and case law.