Objective: We conducted a survey to determine which management options pediatric cardiologists and cardiac surgeons in North America discuss and recommend when counseling parents after the diagnosis of hypoplastic left heart syndrome (HLHS).
Methods: Pediatric cardiologists and cardiac surgeons across North America were asked to complete an anonymous, Internet-based survey about their attitudes and practices regarding the management of HLHS.
Results: We contacted 1621 pediatric cardiologists and surgeons, of whom 749 (46%) completed the survey. When counseling parents of newborns with HLHS, 99.7% of respondents discussed staged palliative surgery, 67% discussed cardiac transplantation, and 62.2% discussed compassionate care without surgery. Only a minority (14.9%) discussed all of those options. Staged palliative surgery was recommended over cardiac transplantation or compassionate care without surgery by 76.2% of respondents. When counseling parents after prenatal diagnosis of HLHS, 98.8% of respondents discussed continuation of pregnancy with staged palliative surgery after birth, 53.5% discussed continuation of pregnancy with cardiac transplantation after birth, 56.9% discussed continuation of pregnancy with compassionate care after birth, and 74.3% discussed termination of pregnancy. Only 36.5% discussed all of those options. Continuation of pregnancy with staged palliative surgery after birth was recommended over the other options by 56% of respondents.
Conclusions: Virtually all North American pediatric cardiologists and cardiac surgeons surveyed discuss a surgical intervention when counseling parents about the care of their child or fetus with HLHS. However, only a minority discuss all options. Most physicians recommend staged palliative surgery for management of HLHS.