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Comparison of Postoperative, In-Hospital Outcomes After Complete Repair of Tetralogy of Fallot Between 22q11.2 Deletion Syndrome and Trisomy 21

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Abstract

22q11.2 deletion syndrome (22q11) and trisomy 21 (T21) are frequently associated with tetralogy of Fallot (TOF). We hypothesized that there are differences in postoperative length of stay (LOS) and occurrence of postoperative interventions after complete repair of TOF when comparing children with 22q11 to those with T21. Using the Pediatric Health Information System, we performed a retrospective cohort study of patients who underwent complete repair of TOF from 2004 to 2019. Three groups were identified: 22q11, T21, and controls (those without a coded genetic syndrome). Outcomes were postoperative LOS and composite occurrence (yes/no) of at least one postoperative intervention. Bivariate and multivariate comparisons were made among groups; odds ratios (ORs) with 95% confidence intervals (CIs) were calculated using the control group as the comparator. There were 6924 subjects (n = 493 22q11, n = 455 T21, n = 5976 controls). In bivariate analysis, 22q11 was associated with a longer LOS compared to T21 (OR 2.37 [2.16, 2.60] vs. 1.25 [1.12, 1.39], p < 0.001), and 22q11 more often underwent postoperative intervention (OR 3.42 [CI 2.56, 4.57] vs. 1.38 [CI 0.91, 2.11]; p < 0.001). In multivariate analysis, 22q11 was also associated with longer LOS (adjusted OR 1.35 [1.26, 1.44] vs. 1.12 [1.04, 1.20]; p < 0.001), but there was no difference in the adjusted odds of postoperative intervention. Children with 22q11 are more likely to experience adverse outcomes after repair of TOF compared to those with T21; the differences are most pronounced for LOS.

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Data Availability

Data from the present study are available from the Children’s Hospital Association, and the Pediatric Health Information System.

Code Availability

Code is available upon request.

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Acknowledgements

No undisclosed authors contributed to the manuscript.

Funding

The present study did not receive funding.

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Authors and Affiliations

  1. Division of General Pediatrics, Department of Pediatrics, University of Arkansas for Medical Sciences and Arkansas Children’s Hospital, Little Rock, AR, USA

    Timothy E. Nissen

  2. Children’s Hospital Association, Lenexa, KS, USA

    Isabella Zaniletti

  3. Division of Pediatric Cardiology, Department of Pediatrics, Stanford University School of Medicine and Lucile Packard Children’s Hospital, Stanford, CA, USA

    R. Thomas Collins II

  4. Division of Pediatric Cardiovascular Surgery, Department of Surgery, University of Arkansas for Medical Sciences and Arkansas Children’s Hospital, Little Rock, AR, USA

    Lawrence E. Greiten

  5. Division of Intensive Care, Department of Pediatrics, University of Arkansas for Medical Sciences and Arkansas Children’s Hospital, Little Rock, AR, USA

    Parthak Prodhan

  6. Division of Cardiology, Department of Pediatrics, University of Arkansas for Medical Sciences and Arkansas Children’s Hospital, Little Rock, AR, USA

    Paul M. Seib & Elijah H. Bolin

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  1. Timothy E. Nissen
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  2. Isabella Zaniletti
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All authors made substantial contributions to the conception of this study, drafted or made revision for important intellectual content, approve of this version being published, and agree to be accountable for all aspects of the work.

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Correspondence to Elijah H. Bolin.

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The authors have no relevant financial or non-financial interests to disclose. The authors declare no conflicts of interest.

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The Institutional Review Board at the University of Arkansas for Medical Sciences deemed the present study not human subjects research.

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De-identified data from the Pediatric Health Information System was used for the present study, and consent to participate was not obtained from subjects.

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Appendix

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Table 5 ICD and CPT codes used to define patient cohort
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Table 6 ICD and CPT codes for postoperative interventions
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Nissen, T.E., Zaniletti, I., Collins, R.T. et al. Comparison of Postoperative, In-Hospital Outcomes After Complete Repair of Tetralogy of Fallot Between 22q11.2 Deletion Syndrome and Trisomy 21. Pediatr Cardiol 43, 290–300 (2022). https://doi.org/10.1007/s00246-021-02683-1

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