A Rare Invader in Complex Heart: Elizabethkingia meningoseptica in a Child With Heterotaxy Syndrome

Introduction: Heterotaxy syndrome is a rare congenital disorder characterized by abnormal left–right organ arrangement, frequently associated with complex congenital heart defects (CCHD). The combination of anatomic anomalies altered lymphatic and splenic function, and the need for repeated surgical interventions creates profound susceptibility to postoperative and opportunistic infections. Elizabethkingia meningoseptica is a non-fermentative, Gram-negative bacillus with unique pathogenic mechanisms. Its ability to survive in chlorinated water, resist multiple disinfectants, and persist within hospital water systems allows it to act as a resilient nosocomial pathogen. It possesses intrinsic resistance to many β-lactam antibiotics and exhibits a strong neurotropic tendency, invading the central nervous system and causing meningitis. In neonates and immunocompromised hosts, this can lead to high mortality and devastating long-term neurologic sequelae if not recognized promptly.


Description: We present the case of a 1-month-old neonate with heterotaxy syndrome, complete atrioventricular canal defect, pulmonary atresia, and major aortopulmonary collateral arteries, status post right ventricle-to-pulmonary artery conduit. The patient was admitted to the PICU with sepsis secondary to necrotizing enterocolitis with bowel perforation. Blood cultures grew Klebsiella pneumoniae and E. meningoseptica. Although initially without neurological symptoms, the patient later developed seizures; brain MRI confirmed findings consistent with meningitis. Given the organism’s neuroinvasive potential, E. meningoseptica was considered the most likely causative pathogen. Early targeted therapy was initiated, and the patient completed the antibiotic course, being discharged without neurologic deficit.


Discussion: This case underscores the need for heightened clinical vigilance for E. meningoseptica in high-risk pediatric cardiac patients. Understanding its environmental resilience, antimicrobial resistance profile, and neurotropic behavior is critical for timely diagnosis, targeted therapy, and prevention of long-term morbidity.