Congenital heart disease (CHD) care has evolved during the past decades. Advances in care have contributed to improved survival among CHD patients. Children with CHD are requiring interventions for non-CHD related medical issues that occur in the general pediatric population. A retrospective review of the Pediatric Health Information System (PHIS) database from January 1, 2004, to July 31, 2023. Discharges of patients with an admitting/principal diagnosis of appendicitis were evaluated and categorized as CHD or non-CHD. A total of 319,228 patients were identified with 1,25,858(39.4%) female, 1,38,966(43.5%) white, and median age of 11[IQR:8-14] years. 708(0.2%) had CHD with 85(12%) of them having a diagnosis consistent with single-ventricle CHD (SV-CHD). In univariate analysis, CHD patients were more likely to undergo conservative treatment (n = 172(24.2%)vs n = 59,358(18.6%)) and less likely to undergo laparoscopic appendectomy (n = 483(68.2%) vs n = 2,35,324(73.8%))(p < 0.001) compared to non-CHD. After adjustment, CHD patients had increased odds of undergoing open appendectomy compared to non-CHD. CHD patients were more likely to have an ICU admission (OR:8.36(95%CI 6.35-10.00),p < 0.001) and had a 77.6%(95%CI 40.89-123.93) increase in length of stay (LOS) (p < 0.001). CHD patients are more likely to have an open appendectomy than non-CHD patients. These findings suggest a distinctive pattern in the care of CHD patients compared to non-CHD. Overall, CHD patients had a more intense level of care with longer LOS and increased ICU admissions. Further work is needed to evaluate drivers of management decisions, the role of conservative treatment with antibiotics alone in the CHD population, and the potential impacts and safety of a laparoscopic approach.