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Anesthetic Challenges in a Patient With TANGO2 Gene Deletion, DiGeorge Syndrome, and Tetralogy of Fallot: A Case
Ivana Wrobleski1, Nischal K Gautam1, Richard M Hubbard1
112340University of Texas Health Science Center at Houston, Houston, TX, USA.
Abstract:
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.
Insights
Mutations in the TANGO2 gene can cause severe metabolic crises, posing anesthetic risks. This case report details managing a patient with TANGO2 deletion, DiGeorge Syndrome, and Tetralogy of Fallot during cardiac intervention.
Area of Science:
- Genetics and rare diseases
- Anesthesiology
- Critical care medicine
Background:
- Mutations in the transport and Golgi organization 2 (TANGO2) gene are associated with neurological decline and acute metabolic crises.
- These crises present with rhabdomyolysis, lactic acidosis, seizures, and cardiac dysfunction, increasing anesthetic risk.
- Optimal management strategies for TANGO2-related crises, particularly in acute and critical care, remain largely undefined.
