Down syndrome and congenital heart disease: perioperative planning and management

Abstract

Approximately 50% of newborns with Down syndrome have congenital heart disease. Non-cardiac comorbidities may also be present. Many of the principles and strategies of perioperative evaluation and management for patients with congenital heart disease apply to those with Down syndrome. Nevertheless, careful planning for cardiac surgery is required, evaluating for both cardiac and noncardiac disease, with careful consideration of the risk for pulmonary hypertension. In this manuscript, for children with Down syndrome and hemodynamically significant congenital heart disease, we will summarize the epidemiology of heart defects that warrant intervention. We will review perioperative planning for this unique population, including anesthetic considerations, common postoperative issues, nutritional strategies, and discharge planning. Special considerations for single ventricle palliation and heart transplantation evaluation will also be discussed. Overall, the risk of mortality with cardiac surgery in pediatric patients with Down syndrome is no more than the general population, except for those with functional single ventricle heart defects. Underlying comorbidities may contribute to postoperative complications and increased length of stay. A strong understanding of cardiac and non-cardiac considerations in children with Down syndrome will help clinicians optimize perioperative care and long-term outcomes.

Keywords: Congenital heart disease; Congenital heart surgery; Down syndrome; Perioperative management; Trisomy 21.

Fig. 1

Kaplan-Meier transplant-free survival plot conditioned on hospital discharge: a after the Stage 1 procedure in patients with DS with functional single ventricle heart defect, b after Glenn procedure in patients with DS with functional single ventricle heart defect, and c after Fontan procedure in patients with DS with functional single ventricle heart defect. DS, Down syndrome; CI, Confidence interval [15]. (Used with permission)