Congenital heart management in Trisomy 13 and 18: Survey of pediatric cardiology providers

Abstract

Abstract Best practices for the care of patients with Trisomy 13 and Trisomy 18 are not well defined. The aim of this work is to identify current perspectives and practices of pediatric cardiac practitioners regarding the care and procedural management of these patients. A survey was distributed to all 412 members of the American Academy of Pediatrics Section on Cardiology and Cardiac Surgery. Respondents were asked about the appropriateness of interventional catheterization and surgical procedures for patients with Trisomy 13/18. Other questions focused on provider perceptions regarding Trisomy 13/18 quality of life, decision making, and other measures of care. 103 respondents met inclusion threshold. With the exception of patent ductus arteriosus stenting, over half agreed or somewhat agreed that various interventional catheter procedures were appropriate for Trisomy 13/18 patients. A majority agreed or somewhat agreed that Risk Adjustment for Congenital Heart Surgery (RACHS) categories 1 (71%) and 2 (58%) procedures were appropriate in Trisomy 13/18, with steep reductions in this sentiment for subsequent RACHS categories. Most respondents did not feel that extracorporeal membrane oxygenation is reasonable in this population. 59% reported that their institution has no written or generally agreed upon policies for the care of this population. Overall, findings demonstrate variability regarding the perceived appropriateness of specific interventions for patients with Trisomy 13/18, with most providers feeling that lower-risk interventions may be appropriate. Providers, pediatric heart centers, and families may benefit from better defined institutional approaches to the management of these patients.