Introduction Postpump chorea is a rare but potentially disabling movement disorder that arises days to weeks after cardiopulmonary bypass, most often following deep hypothermic circulatory arrest in pediatric patients undergoing repair of complex congenital heart defects. Clinically, postpump chorea presents as involuntary, nonrhythmic, choreiform, and athetotic movements after an initial asymptomatic period. Methods We present a detailed case of a two‐year‐old male with an interrupted aortic arch and a large ventricular septal defect who developed persistent chorea four days after deep hypothermic circulatory arrest repair at 18°C for 145 min. A focused literature review was conducted in May 2025 via PubMed, MEDLINE, Scopus, and Google Scholar. Results Our patient’s structural neuroimaging and metabolic/autoimmune workups were unremarkable. Initial treatment with clonazepam yielded partial improvement; addition of haloperidol provided further relief but did not fully resolve chorea, which persisted intermittently at the last follow‐up. Antegrade cerebral perfusion through the innominate artery was not used due to anatomic constraints and the need for complete arch reconstruction. Literature review confirms postpump chorea onset typically 3–14 days postcardiopulmonary bypass, frequent normal findings on CT/MRI, and variable responses to benzodiazepines, dopamine antagonists, and VMAT‐2 inhibitors. Identified risk factors include prolonged deep hypothermic circulatory arrest time, lower target temperatures, pH‐stat management, and cyanotic shunts. Conclusion Postpump chorea demands high clinical vigilance despite normal imaging. Prevention through optimized cardiopulmonary bypass techniques, including moderate hypothermia, controlled rewarming, pH‐stat blood gas management, emboli filtration, and real‐time cerebral monitoring, is paramount. First‐line medical therapy (clonazepam and haloperidol) should be initiated promptly, with VMAT‐2 inhibitors or corticosteroids reserved for refractory cases. Future multicenter studies are needed to refine neuroprotective strategies and define long‐term neurodevelopmental outcomes.
Khalek et al. (Thu,) studied this question.