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Objectives: We analyzed the impact of postoperative necrotizing enterocolitis (NEC) after cardiac surgery in neonatal age on neurodevelopmental (ND) outcome at 1 year of age., Methods: Using data from the Swiss Neurodevelopmental Outcome Registry for Children with Congenital Heart Disease (ORCHID), we analyzed perioperative variables including postoperative NEC (Bell's stage ≥2) and 1-year ND outcome (Bayley III)., Results: The included patients ( n  = 101) had congenital heart disease (CHD), categorized as follows: 77 underwent biventricular repair for CHD with two functional chambers, 22 underwent staged palliation until the Fontan procedure for CHD with single ventricle physiology ( n  = 22), or 4 underwent single ventricle palliation or biventricular repair for borderline CHD ( n  = 4). Neonatal cardiopulmonary bypass (CBP) surgery was performed at a median age (IQR) of 8 (6) days. NEC occurred in 16 patients. Intensive care unit (ICU) length of stay (LOS) and the total duration of the hospitalization were longer in children with NEC than those in others (14 with vs. 8 days without NEC, p  < 0.05; 49 with vs. 32 days without NEC, p  < 0.05). The Bayley III scores of the analyzed patients determined at an age of 11.5 ± 1.5 months showed cognitive (CCS) (102.2 ± 15.0) and language scores (LCS) (93.8 ± 13.1) in the normal range and motor composite scores (MCS) (88.7 ± 15.9) in the low-normal range. After adjusting for socioeconomic status and CHD type, patients with NEC had lower CCS scores [ β  = -11.2 (SE 5.6), p  = 0.049]. Using a cumulative risk score including NEC, we found a higher risk score to be associated with both lower CCS [ β  = -2.8 (SE 1.3), p  = 0.030] and lower MCS [ β  = -3.20 (SE 1.3), p  = 0.016]., Conclusions: Postoperative NEC is associated with longer ICU and hospital LOS and contributes together with other complications to impaired ND outcome at 1 year of age. In the future, national and international patient registries may provide the opportunity to analyze large cohorts and better identify the impact of modifiable perioperative risk factors on ND outcome., Clinical Trial Registration: ClinicalTrials.gov identifier: NCT05996211., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (© 2024 Knirsch, De Silvestro, Rathke, L'Ebraly, Natterer, Schneider, Sekarski, Latal, Borradori-Tolsa, Bouhabib, Fuhrer Kradolfer, Glöckler, Hutter, Pfluger, Kaiser, Polito, Kelly-Geyer, von Rhein and the Swiss ORCHID group.)