Background: Cyanotic congenital heart disease (CCHD) poses significant perioperative challenges, particularly
during emergency neurosurgical procedures. Double outlet right ventricle (DORV) is a rare cyanotic congenital heart
disease that complicates anesthetic management due to chronic hypoxemia and right-to-left shunting.
Case Presentation: We report the case of a 5-year-old boy with uncorrected DORV who sustained severe traumatic
brain injury after a fall. Head CT revealed a large right temporoparietooccipital epidural hematoma with subdural
and subarachnoid hemorrhage and a 1.7 cm midline shift. Emergency craniotomy was performed under general
anesthesia with invasive hemodynamic monitoring and cautious ventilatory management to balance cerebral and
cardiac demands. Although intraoperative hemodynamics remained stable and the hematoma evacuation was
technically successful, the patient developed persistent postoperative hypoxemia and multiorgan failure, resulting in
death on postoperative day three.
Despite a technically successful evacuation of the hematoma and stable intraoperative parameters, the patient’s
postoperative course was complicated by persistent hypoxemia due to right-to-left shunting, culminating in
multiorgan failure and death after three days of intensive care. This case underscores the profound anesthetic and
surgical challenges posed by the coexistence of severe traumatic brain injury and uncorrected cyanotic congenital
heart disease, highlighting the importance of multidisciplinary collaboration, tailored perioperative strategies, and
the urgent need for early cardiac corrective interventions to improve outcomes in similar high-risk scenarios.
Conclusion:This case demonstrates the profound anesthetic and surgical challenges of emergency neurosurgery in
pediatric patients with uncorrected cyanotic congenital heart disease. Effective management requires
multidisciplinary collaboration, individualized anesthetic strategies, and early cardiac correction to improve survival
in such high-risk scenarios.
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