Key result
Surgical repair of an atrial septal defect under cardiopulmonary bypass was performed uneventfully without metabolic crisis or postoperative complications in a 4-year-old girl with 3-MCC deficiency.
Why the study?
3-MCC deficiency may lead to metabolic decompensation under stress, but outcomes of elective surgery requiring cardiopulmonary bypass are unknown.
Does surgical ASD repair under CPB cause metabolic decompensation in a patient with 3-MCC deficiency?
Case Report (n=1)
No
Does surgical ASD repair under CPB cause metabolic decompensation in a patient with 3-MCC deficiency?
Surgical ASD repair under cardiopulmonary bypass can be performed safely without triggering metabolic decompensation in a patient with asymptomatic 3-MCC deficiency.
Successful CPB in asymptomatic 3-MCC deficiency; leaves open evidence-based perioperative protocols for this rare disorder.
3-Methylcrotonyl-CoA carboxylase (3-MCC) deficiency is a rare autosomal recessive disease of leucine catabolism. 3-MCC deficiency may lead to metabolic decompensation under stress; however, outcomes of elective surgery requiring cardiopulmonary bypass (CPB) are unknown. We report a 4-year-old girl with asymptomatic 3-MCC deficiency and atrial septal defect (ASD) who's undergone surgical ASD repair under CPB. She was otherwise normal developmentally and medically. Although patients with 3-MCC may face metabolic crises, the ASD repair under CPB was uneventful.
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Alhamoud et al. (2024) conducted a case report in 3-Methylcrotonyl-CoA Carboxylase Deficiency and Atrial Septal Defect (n=1). Surgical ASD repair under cardiopulmonary bypass was evaluated on Postoperative complications and metabolic crisis. Surgical repair of an atrial septal defect under cardiopulmonary bypass was performed uneventfully without metabolic crisis or postoperative complications in a 4-year-old girl with 3-MCC deficiency.
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