Abstract
BACKGROUND: Patients with trisomy 21 (T21) often have elevated pulmonary vascular resistance, which may result in a suboptimal cavopulmonary connection (CPC) after a Glenn or Fontan operation. The purpose of this study was to assess, in a nationwide, multiinstitution cohort of patients with CPC, the impact of T21 on patient morbidity, mortality, and resource use. METHODS: A total of 23,271 pediatric patients with CPC (2004 to 2019) at 50 US hospitals were evaluated using the Pediatric Health Information System database. Univariable and multivariable regression analyses were used to assess risk-adjusted associations between Down syndrome and other risk factors and postoperative measures of morbidity, mortality, lengths of stay, and cost of hospitalization. RESULTS: The overall prevalence of T21 among patients who had undergone Glenn and Fontan procedures was 1.5% (199 of 13,268) and 0.8% (78 of 1003), respectively. Among both CPC cohorts, T21 status significantly increased unadjusted mortality, hospital lengths of stay, and total costs of hospitalization compared with the non-T21 CPC cohort (all P < .001). Patients with T21 also had a higher incidence of prolonged mechanical ventilation compared with patients without T21 in both Glenn and Fontan groups (P < .001). Multivariable regression analysis further estimated that patients with T21 are associated with a 5.5-fold increase in mortality (P < .001) compared with patients without T21. Finally, patients with T21 had increased long-term mortality compared with their peers. CONCLUSIONS: T21 significantly increases risk-adjusted morbidity, inpatient mortality, long-term mortality, and resource use after cavopulmonary connections. Further investigation is needed to clarify modifiable patient-level and center-specific risk factors to improve outcomes for patients with T21.