Mutations of the transport and Golgi organization 2 (TANGO2) genes are linked with both long-term neurological decline and acute metabolic crises during stress, leading to significant anesthetic risk. Crises are marked by rhabdomyolysis, lactic acidosis, seizures, cardiac dysfunction, and dysrhythmias. Much is unknown about optimal management of this condition, especially in the acute and critical care settings. The following report describes the anesthetic challenges of a patient with simultaneous TANGO2 gene deletion, DiGeorge Syndrome, and Tetralogy of Fallot, who presented for an interventional cardiac procedure with the goal of metabolic crisis-avoidance and facilitation of safe but expeditious recovery and discharge home.
Keywords: cardiac anesthesia; children; genetic determinants; pediatric intensive care; perioperative glucose control.