The Burden of Congenital Heart Disease and Urogenital Lesions in a National Cohort of Hirschsprung Patients

Hirschsprung's disease (HSCR) is often associated with other congenital and chromosomal defects. This study aimed to describe the prevalence of congenital heart disease (CHD) and congenital urogenital lesions in children with HSCR, with and without Trisomy 21 and other associated anomalies, to...

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Bibliographic Details
Published in:Journal of pediatric surgery 2023-11, Vol.58 (11), p.2165-2170
Main Authors: Salvi, Pooja S., Cowles, Robert A.
Format: Article
Language:English
Subjects:
Congenital heart disease
Hirschsprung disease
Trisomy 21
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Summary:Hirschsprung's disease (HSCR) is often associated with other congenital and chromosomal defects. This study aimed to describe the prevalence of congenital heart disease (CHD) and congenital urogenital lesions in children with HSCR, with and without Trisomy 21 and other associated anomalies, to guide appropriate screening. The Pediatric Health Information System was queried for patients with HSCR who underwent surgical treatment between 2016 and 2021. The prevalence of CHD, congenital urogenital lesions, Trisomy 21 and other congenital syndromes were calculated. Multivariable regression modeling was used to identify predictors of postoperative intensive care unit (ICU) admission and postoperative length of stay (LOS). Of 2021 HSCR patients at 47 children's hospitals, 264 (13.1%) had CHD, 244 (12.1%) had Trisomy 21, and 103 (5.1%) had a congenital urogenital lesion. The prevalence of CHD (49.6 vs. 8.1%, P 
ISSN:0022-3468
1531-5037
DOI:10.1016/j.jpedsurg.2023.06.014