A Perfect Storm in Paediatric Anaesthesia: Uncorrected Transposition of Great Arteries with Goldenhar Syndrome Undergoing Non-Cardiac Surgery

Abstract

Background: Coexisting uncorrected dextro-transposition of the great arteries (d-TGA) and Goldenhar syndrome is exceptionally rare and presents significant perioperative risk, particularly in non-cardiac surgery.

Case Presentation:Case presentation: Prior to cardiac surgery, a 5-year-old boy with d-TGA, a large atrial septal defect, patent ductus arteriosus, and Goldenhar syndrome craniofacial features was scheduled for full-mouth rehabilitation. On room air, the baseline oxygen saturation was between 62 and 72%. A thorough airway plan was created in anticipation of a challenging airway. Systemic vascular resistance was maintained during induction with ketamine and fentanyl, and tracheal intubation was accomplished successfully on the first try with a C-MAC D-blade video laryngoscope. Sevoflurane in 50% oxygen-air was used to maintain anesthesia, avoiding substances that could raise pulmonary vascular resistance. Intraoperative oxygen saturation improved to 92%, and hemodynamics stayed steady. After a smooth extubation, the patient was observed in the pediatric intensive care unit.

Conclusion: Careful preoperative assessment, multidisciplinary coordination, and advanced airway planning are essential in managing rare combinations of cyanotic congenital heart disease and craniofacial anomalies. Video laryngoscopy and ketamine-based induction can optimise both airway safety and cardiovascular stability in such high-risk paediatric patients.