Congenital Heart Surgery Outcomes in Down Syndrome: Analysis of a National Clinical Database

Abstract

OBJECTIVE: We describe patient characteristics and postoperative morbidity and mortality rates for patients with Down syndrome undergoing congenital heart disease surgery. METHODS: This retrospective cohort study used the Society of Thoracic Surgeons Congenital Heart Surgery Database to compare patient characteristics and postoperative outcomes for patients (0–18 years) with or without Down syndrome who underwent surgery in 2000–2008. RESULTS: A total of 45 579 patients (4350 patients with Down syndrome and 41 229 without Down syndrome) were included (median age: 7 months [interquartile range [IQR]: 47 days to 4 years]; 56% male). Patients with Down syndrome were younger at surgery, with the exception of those undergoing tetralogy of Fallot repair or atrioventricular septal defect repair. Mortality rates for patients with or without Down syndrome did not differ significantly. Lengths of stay were prolonged for patients with Down syndrome undergoing atrial septal defect closure (median: 4 days [IQR: 3–5 days] vs 3 days [IQR: 2–4 days]; P < .0001), ventricular septal defect closure (median: 5 days [IQR: 4–8 days] vs 4 days [IQR: 3–6 days]; P < .0001), or tetralogy of Fallot repair (7 days [IQR: 5–10 days] vs 6 days [IQR: 5–9 days]; P < .001) and were associated with postoperative respiratory and infectious complications. Patients with Down syndrome undergoing ventricular septal defect closure had a higher rate of heart block requiring pacemaker placement (2.9% vs 0.8%; P < .0001). CONCLUSION: In this large, contemporary cohort, Down syndrome did not confer a significant mortality risk for the most common operations; however, postoperative morbidity remained common.