Discussing Nonsurgical Care With Parents of Newborns With Hypoplastic Left Heart Syndrome

Hypoplastic left heart syndrome (HLHS) is a devastating congenital cardiac defect. Currently, there are three management options for children with HLHS: the Norwood procedure, cardiac transplantation, and comfort care without surgery. In a recent publication, my colleagues and I discussed our findings that many physicians do not disclose all three management options with parents of affected neonates [Kon AA, Ackerson L, Lo B: How pediatricians counsel parents when no “best-choice” treatment exists: lessons to be learned from the hypoplastic left heart syndrome. Arch Pediatr Adolesc Med 158:436-441, 2004]. When I have presented this research at conferences, many audience members have debated the pros and cons of offering nonsurgical care to parents. There is clearly a group of practitioners who feel very strongly that comfort care without surgery should never be presented to parents. I, however, disagree. I believe that all parents must be presented with all three management options. In the following essay, I will first provide background on HLHS, describe the two surgical options and discuss the current published data on outcomes, outline the four arguments generally put forth by those who feel that nonsurgical care should not be offered to parents, and finally, rebut each of these arguments and explain why all parents of children with HLHS must be told about comfort care without surgery as a reasonable choice.