Fulminating Enterocolitis in Term Neonates with Major Cardiac Defects: A Review of Hypoxic Mucosal Shedding and Postoperative Risk

Abstract

Objectives: To review the pathophysiology and management of fulminating Enterocolitis (EC) in term neonates with major cardiac defects.

Background: Unlike classic NEC in preterm infants, cardiac EC is a "vascular catastrophe" driven by mesenteric low-flow states and anatomical vulnerabilities.

Design: Narrative review of published literature, clinical guidelines, and surgical series across pediatric cardiology, neonatal and pediatric surgery, and neonatal/perinatal medicine.

Data Sources: MEDLINE, Embase, Cochrane Library, and major pediatric surgical and cardiology and cardiothoracic surgery society guidelines.

Eligibility Criteria: Studies describing structural, functional, or inflammatory and infective lesions of the fulminating necrotising enterocolitis in neonatal term cardiac patients from birth to 1 month, with emphasis on conditions requiring surgical assessment or intervention.

Results: The clinical course of Enterocolitis (EC) in neonates with Congenital Heart Disease (CHD) is characterized by a significantly higher burden of disease compared to classical neonatal NEC. High-risk lesions include HLHS and Coarctation. Key risks include the watershed areas of marginal colonic supply (Griffith’s, Sudeck’s, and ileocecal points). Furthermore, the terminal reach of vasa recta makes the antimesocolic area a zone of relative ischaemia. Postoperative ileus exacerbates this by compressing microvascular flow, leading to rapid hypoxic mucosal shedding.

Conclusions: Fulminating Enterocolitis (EC) in term neonates with congenital heart disease (CHD) represents a distinct vascular phenotype, diverging from the dysbiosis-driven Necrotising Enterocolitis (NEC) of prematurity. This condition is characterised by segmental ischaemia at anatomical watershed zones and rapid progression to transmural infarction. Mortality ranges from 24% to 50%, with single ventricle physiology reaching up to 97%. Early Point-of-Care Ultrasound (POCUS) detection of mucosal shedding and mural avascularity offers a proactive diagnostic alternative to late-stage radiographic signs. Surgical management prioritises perfusion-preserving strategies and anatomical sparing. This manuscript synthesises clinical outcomes, imaging findings, and anatomical risk factors, advocating for a paradigm shift in diagnostic and operative management. Early recognition of these anatomical triggers via Point-of-Care Ultrasound (POCUS) is critical to reducing the high morbidity and mortality in this population.

Keywords: Enterocolitis; Necrotizing; Heart Defects; Congenital; Watershed Areas; Fulminating Enterocolitis; Congenital Heart Disease; Hypoxic Mucosal Shedding; Anatomical Watershed Zones; Antimesocolic Ischaemia; Postoperative Ileus; Bacterial Translocation; Point-of-Care Ultrasound (POCUS); Low Cardiac Output Syndrome (LCOS)

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