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Persistent chorea following cardiac surgery for congenital heart disease
S Osari1, H Muranaka, T Kojima
1Department of Pediatrics, Hirosaki, University, Japan.
Insights
A child developed chorea, a movement disorder, after heart surgery in Japan. This case highlights cardiac surgery as a potential trigger for such conditions, with pimozide proving effective for treatment.
Area of Science:
- Pediatric Neurology
- Cardiovascular Surgery
- Movement Disorders
Background:
- Total anomalous pulmonary venous return (TAPVR) is a complex congenital heart defect requiring surgical correction.
- Hypothermia is often employed during cardiopulmonary bypass for pediatric cardiac surgery to reduce metabolic demand and protect organs.
- Post-operative neurological complications can occur following cardiac surgery, particularly in infants.
Observation:
- A 6-year-old girl presented with chorea, irritability, dysphagia, hypotonia, and ophthalmoplegia seven days after undergoing radical surgery for TAPVR at 11 months of age.
- The neurological symptoms, including chorea, persisted despite gradual improvement of other signs.
- This represents the first reported case of post-cardiac surgery chorea in Japan.
Findings:
- The clinical presentation suggests a potential link between cardiac surgery performed under hypothermia and the development of chorea.
- The persistent nature of chorea indicates a possible subcortical or basal ganglia insult.
- Pharmacological management was explored, with pimozide demonstrating efficacy in reducing chorea symptoms without adverse effects.
Implications:
- Cardiac surgery, especially when involving hypothermia, should be considered a potential etiological factor in pediatric movement disorders like chorea.
- Further research is warranted to elucidate the precise mechanisms linking cardiac surgery to chorea.
- Pimozide may be a viable therapeutic option for managing post-surgical chorea in pediatric patients.
Abstract:
We describe a 6 year old girl with chorea following cardiac surgery, the first such report in Japan. The radical operation for total anomalous pulmonary venous return was carried out at the age of 11 months under hypothermia. Seven days after the operation, a sudden onset of irritability, dysphagia, chorea, generalized, hypotonia, and complete external ophthalmoplegia were seen. These symptoms diminished gradually, but chorea remained. We speculated that the cause of chorea arose from the cardiac surgery under hypothermia. It is necessary to consider 'cardiac surgery' as one of the triggers of certain movement disorders including chorea. We tried treatment with haloperidol, pimozide, and several other drugs; only pimozide was effective in decreasing chorea without any side-effects.