Jessie Mei Lim1, Prashob Porayette2, Davide Marini2, Vann Chau3,4, Stephanie H Au-Young3,5, Amandeep Saini2, Linh G Ly4, Susan Blaser6, Manohar Shroff7,6, Helen M Branson6, Renee Sananes8,9, Edward J Hickey10, J William Gaynor11, Glen Van Arsdell10, Steven P Miller8,3, Mike Seed8,2. 1. Department of Physiology (J.M.L.), University of Toronto, Canada. 2. Division of Cardiology (P.O., D.M., A.S., M. Seed), The Hospital for Sick Children, Toronto, Canada. 3. Division of Neurology (V.C., S.H.A.-Y., S.P.M.), The Hospital for Sick Children, Toronto, Canada. 4. Division of Neonatology (V.C., L.G.L.), The Hospital for Sick Children, Toronto, Canada. 5. Neurosciences and Mental Health, The Hospital for Sick Children Research Institute, Toronto, Canada (S.H.A.-Y.). 6. Department of Diagnostic Imaging (S.B., M. Shroff, H.M.B.), The Hospital for Sick Children, Toronto, Canada. 7. Department of Medical Imaging (M. Shroff), University of Toronto, Canada. 8. Department of Pediatrics (R.S., S.P.M., M. Seed), University of Toronto, Canada. 9. Labatt Family Heart Centre (R.S.), The Hospital for Sick Children, Toronto, Canada. 10. Division of Cardiovascular Surgery (E.J.H., G.V.A.), The Hospital for Sick Children, Toronto, Canada. 11. Department of Surgery, Children's Hospital of Philadelphia, PA (J.W.G.).
Abstract
BACKGROUND: Brain injury, impaired brain growth, and long-term neurodevelopmental problems are common in children with transposition of the great arteries. We sought to identify clinical risk factors for brain injury and poor brain growth in infants with transposition of the great arteries undergoing the arterial switch operation, and to examine their relationship with neurodevelopmental outcome. METHODS: The brains of 45 infants with transposition of the great arteries undergoing surgical repair were imaged pre- and postoperatively using magnetic resonance imaging. Brain weight z scores were calculated based on brain volume and autopsy reference data. Brain injury scores were determined as previously described. Neurodevelopment was assessed at 18 months using the Bayley-III scores of infant development. The relationships between clinical variables, brain injury, perioperative brain growth, and 18-month Bayley-III scores were analyzed. RESULTS: On preoperative imaging, moderate or severe white matter injury was present in 10 of 45 patients, whereas stroke was seen in 4 of 45. A similar prevalence of injury was seen on postoperative imaging, and we were unable to identify any clinical risk factors for brain injury. Brain weight z scores decreased perioperatively in 35 of 45 patients. The presence of a ventricular septal defect ( P=0.009) and older age at surgery ( P=0.007) were associated with impaired perioperative brain growth. When patients were divided into those undergoing surgery during the first 2 weeks of life (32/45) versus those being repaired later (13/45), infants repaired later had significantly worse perioperative brain growth (late repair postoperative brain weight z = -1.0±0.90 versus early repair z = -0.33±0.64; P=0.008). Bayley-III testing scores fell within the normal range for all patients, although age at repair ( P=0.03) and days of open chest ( P=0.03) were associated with a lower composite language score, and length of stay was associated with a lower composite cognitive score ( P=0.02). CONCLUSIONS: Surgery beyond 2 weeks of age is associated with impaired brain growth and slower language development in infants with transposition of the great arteries cared for at our center. Although the mechanisms underlying this association are still unclear, extended periods of cyanosis and pulmonary overcirculation may adversely impact brain growth and subsequent neurodevelopment.
BACKGROUND:Brain injury, impaired brain growth, and long-term neurodevelopmental problems are common in children with transposition of the great arteries. We sought to identify clinical risk factors for brain injury and poor brain growth in infants with transposition of the great arteries undergoing the arterial switch operation, and to examine their relationship with neurodevelopmental outcome. METHODS: The brains of 45 infants with transposition of the great arteries undergoing surgical repair were imaged pre- and postoperatively using magnetic resonance imaging. Brain weight z scores were calculated based on brain volume and autopsy reference data. Brain injury scores were determined as previously described. Neurodevelopment was assessed at 18 months using the Bayley-III scores of infant development. The relationships between clinical variables, brain injury, perioperative brain growth, and 18-month Bayley-III scores were analyzed. RESULTS: On preoperative imaging, moderate or severe white matter injury was present in 10 of 45 patients, whereas stroke was seen in 4 of 45. A similar prevalence of injury was seen on postoperative imaging, and we were unable to identify any clinical risk factors for brain injury. Brain weight z scores decreased perioperatively in 35 of 45 patients. The presence of a ventricular septal defect ( P=0.009) and older age at surgery ( P=0.007) were associated with impaired perioperative brain growth. When patients were divided into those undergoing surgery during the first 2 weeks of life (32/45) versus those being repaired later (13/45), infants repaired later had significantly worse perioperative brain growth (late repair postoperative brain weight z = -1.0±0.90 versus early repair z = -0.33±0.64; P=0.008). Bayley-III testing scores fell within the normal range for all patients, although age at repair ( P=0.03) and days of open chest ( P=0.03) were associated with a lower composite language score, and length of stay was associated with a lower composite cognitive score ( P=0.02). CONCLUSIONS: Surgery beyond 2 weeks of age is associated with impaired brain growth and slower language development in infants with transposition of the great arteries cared for at our center. Although the mechanisms underlying this association are still unclear, extended periods of cyanosis and pulmonary overcirculation may adversely impact brain growth and subsequent neurodevelopment.
Entities:
Keywords:
arterial switch operation; brain; growth & development; surgery; transposition of great vessels
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