Background: 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.
Methods: 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).
Results: 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 < 0.001) and of undergoing a cardiac intervention with associated CHD (40.5 vs. 23.1%, P = 0.002) were higher in patients with Trisomy 21 compared to those without. CHD was associated with an increased likelihood of postoperative ICU admission (OR: 1.6, 95% CI: 1.1, 2.2) and greater postoperative LOS (IRR: 2.6, 95% CI: 2.6, 2.7), irrespective of Trisomy 21 diagnosis.
Conclusions: The prevalence of CHD among HSCR patients (13.1%) was higher than previously reported, and CHD patients required more resource-intensive care after pull-through. While Trisomy 21 was associated with higher rates of CHD and cardiac intervention, 8.1% of HSCR patients without Trisomy 21 had CHD. Screening echocardiogram should be considered in all children diagnosed with HSCR, as CHD may influence perioperative risk stratification. However, screening renal ultrasound may have limited utility given the low prevalence of urogenital lesions in this population.
Level of evidence: Level III.
Keywords: Congenital heart disease; Hirschsprung disease; Trisomy 21.
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