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Background and Aims Tracheoesophageal fistula (TEF) is a congenital anomaly often observed with other significant developmental defects, such as those characterised by the VACTERL association, which includes vertebral defects, anal atresia, cardiac defects, renal anomalies, and limb deformities. Management of Double Outlet Right Ventricle (DORV) adds complexity, requiring a detailed understanding of haemodynamics influenced by the alignment of major arteries, connections between ventricles, and obstructions in the outflow tract. This case report examines the anaesthetic management of a 2-day-old, 32-week preterm female neonate with DORV and pulmonary stenosis (PS), who required surgical intervention for TEF.

Methods Initial management included stabilisation on nasal CPAP followed by diagnostic assessments confirming DORV and TEF. The anaesthetic approach was tailored to maintain systemic vascular resistance (SVR) and minimise pulmonary vascular resistance (PVR), essential for preventing cyanotic spells. IV ketamine was selected over inhalational agents to expedite induction and to maintain SVR. In response to intraoperative hypotension episodes, volume and SVR were increased using alpha agonists such as phenylephrine.

Results Surgical correction of TEF was achieved through meticulous anaesthetic management, including maintaining euvolaemia and air bubbles hygiene. Monitoring and adjusting perioperative conditions were crucial to prevent hypoxia, hypercapnia, and acidosis, thereby managing PVR and avoiding cyanotic spells.

Conclusions This case underscores the intricate anaesthetic strategies required in managing neonate with DORV and TEF, emphasising the importance of understanding applied physiology and pharmacology. Effective management involves a careful balance of SVR and PVR, preventing intraoperative complications, and ensuring stable haemodynamics throughout the surgical procedure.