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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