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Perioperative management of a parturient with long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency
Abstract Number: RF7A10-119
Abstract Type: Case Report Case Series
Long chain 3-hydroxyacyl CoA dehydrogenase deficiency (LCHAD) is a rare, autosomal-recessive fatty acid oxidation disorder. Due to this enzyme deficiency, the patient is incapable of metabolizing long chain fatty acids efficiently and is unable to fast. LCHAD is a potentially life-threatening condition especially when patients undergo stress, illness or processes that required high metabolic demand. Patients with LCHAD can also have cardiomyopathy and rhythm disorders, especially in the setting of acute onset chest pain (1).
There is very little data on pregnancy, pregnancy related complications and perioperative management of females with long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD). During pregnancy the caloric intake should be gradually increased by increasing both carbohydrate (simple and complex) and medium chain fatty acid (MCT) intake. The mainstay of treatment is the use of intravenous (IV) glucose as an energy source, treatment of cardiac rhythm disturbances, and monitoring of rhabdomyolysis (1).
Our case involves a patient admitted for nausea and vomiting in setting of medication non-compliance. Her pregnancy was complicated by poor caloric intake, prolonged QTc, rhabdomyolysis and transient transaminitis. Our patient was a 22 year old F G1P1001 @ 37 weeks with LCHAD deficiency, blindness due to pigmentary retinopathy, major depression, and acquired long QT syndrome with torsades. On admission, her CK and transaminases were found to be elevated. Her initial symptoms began a week before admission with lower leg weakness/cramping that progressively got worse and later developed nausea/vomiting two days prior to admission. Our patients symptoms were likely due to her pregnancy placing a high metabolic demand on her body which was causing her to go into a catabolic state. Because of her lack of appropriate caloric intake and decreased intake of MCT oil she was at higher risk for rhabdomyolysis even in absence of exercise or exertion.
During her admission her caloric status was optimized by providing high calorie content fluids (10% dextrose) and providing MCT supplementation. Important anesthetic considerations included limiting fasting periods, reducing surgical stress, avoiding large amounts or prolonged propofol infusions and avoiding QT prolongating medications (2). She was managed expectantly but during her stay she delivered via emergent cesarean delivery due to fetal decelerations. Her IV dextrose infusion was continued intraoperatively and a spinal anesthetic was performed without post partum complications.
1. Welsink-Karssies et al 2017. Very Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency and Perioperative Management in Adult Patients. JIMD Rep. 2017;34-49-54
2. Martina et al., 2014. Use of propofol for short duration procedures in children with long chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) or trifunctional protein (TFP) deficiencies. Mol Genet Metab. 2014 June ; 112(2): 139–142.