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Down Syndrome Patients Undergoing Cavopulmonary Connections Have Increased Mortality And Morbidity: A Nationwide, Multi-institutional Study
Philip Allen, Michael Kwon, Brett Anderson, Emile Bacha, Damien LaPar.
Columbia University College of Physicians and Surgeons, New York, NY, USA.

Objectives: Trisomy 21 (T21) patients have elevated pulmonary vascular resistance and may have greater risk after cavopulmonary connections (CPC) (Glenn and Fontan). This study's aim was to assess in a nationwide, multi-institution CPC cohort the impact of T21 on inpatient and long-term morbidity, mortality, and resource utilization.
Methods:A total of 23,271 CPC pediatric patients (2004 2019) at over 40 U.S. hospitals were evaluated using the Pediatric Health Information System (PHIS) administrative database. Univariable and multivariable regression analyses were used to assess risk-adjusted associations between T21 and morbidity, mortality, lengths of stay (LOS) and hospitalization costs.
Results:The overall prevalence of T21 for Glenn and Fontan was 1.5% (199/13,268) and 0.8% (78/1,003). T21 significantly increased unadjusted mortality, hospital LOS and total hospitalization costs (all P < 0 .001). T21 patients also incurred a higher incidence of prolonged mechanical ventilation (P < 0.001). Multivariable regressions further estimated that T21 is associated with an 8.4-fold increase in mortality (P < 0 .001) and increased ICU LOS, total LOS, overall costs (all P <0.01). Lastly, T21 patients had increased long-term mortality compared to their peers, especially in the 2 years following cavopulmonary connections (Figure).
Conclusion(s):Trisomy 21 significantly increases risk-adjusted morbidity, inpatient mortality, long-term mortality and resource utilization following cavopulmonary connections. Further investigation is needed to clarify modifiable patient-level risk factors to improve outcomes for T21 patients.


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