Left temporal plane growth predicts language development in newborns with congenital heart disease.

Congenital heart defects are the most common congenital anomalies, accounting for a third of all congenital anomaly cases. While surgical correction dramatically improved survival rates, the lag behind normal neurodevelopment appears to persist. Deficits in higher cognitive functions are particularly common, including developmental delay in communication and oral-motor apraxia. It remains unclear whether the varying degree of cognitive developmental delay is reflected in variability in brain growth patterns. To answer this question, we aimed to investigate whether the rate of regional brain growth is correlated with later life neurodevelopment. Forty-four newborns were included in our study, of whom 33 were diagnosed with dextro-transposition of the great arteries and 11 with other forms of severe congenital heart defects. During the first month of life, neonates underwent corrective or palliative cardiovascular bypass surgery, pre- and postoperative cerebral MRI were performed 18.7 ± 7.03 days apart. MRI was performed in natural sleep on a 3.0 T scanner using an 8-channel head coil, fast spin-echo T2-weighted anatomical sequences were acquired in three planes. Based on the principles of deformation-based morphometry, we calculated brain growth rate maps reflecting average daily growth occurring between pre- and postoperative brain images. An explorative, whole-brain, threshold-free cluster enhancement analysis revealed strong correlation between the growth rate of the Heschl's gyrus, anterior planum temporale and language score at 12 months of age, corrected for demographic variables (P = 0.018, t = 5.656). No significant correlation was found between brain growth rates and motor or cognitive scores. Post hoc analysis showed that the length of hospitalization interacted with this correlation, longer hospitalization resulted in faster enlargement of the internal CSF spaces. Our longitudinal cohort study provides evidence for the early importance of left-dominant perisylvian regions in auditory and language development before direct postnatal exposure to native language. In congenital heart disease patients, the perioperative period results in a critical variability of brain growth rate in this region, which is a reliable neural correlate of language development at 1 year of age.

Significance of patient categorization for perioperative management of children with tetralogy of Fallot, with special regard to co-existing malformations.

BACKGROUND: The aim of our study was to facilitate perioperative calculation of potential risk factors on the outcome of corrective surgery for children with tetralogy of Fallot. METHODS: The medical records of 81 (44 female and 37 male) out of a total of 87 patients undergoing complete surgical repair of tetralogy of Fallot between 1988 and 2004 at the Children's Hospital of the Johannes Gutenberg University of Mainz were reviewed. PATIENTS were divided into four categories, depending on the severity of pulmonary stenosis and cyanosis, as well as on the type of pulmonary circulation. RESULTS: Additional malformations did not affect mortality rates, but did directly affect the number of pleural effusions, time of epinephrine administration, duration of surgery, bypass, and ischemia, as well as length of hospitalization and intensive care unit treatment. In contrast to longer periods of extracorporeal circulation and ischemia during surgery, which are directly related not only to more complex anatomical situations but also to higher mortality and complication rates, the much-debated question of age at surgery had no influence either on the surgical approach itself or on the post-operative outcome. CONCLUSIONS: Our patient categorization, and evaluation of potential pre-operative risk factors and intraoperative parameters, should prove useful for the future planning and execution of therapeutic procedures in institutions around the world.