Executive function and theory of mind in school-aged children after neonatal corrective cardiac surgery for transposition of the great arteries.

AIM: cardiac malformations resulting in cyanosis, such as transposition of the great arteries (TGA), have been associated with neurodevelopmental dysfunction. The purpose of this study was to assess, for the first time, theory of mind (ToM), which is a key component of social cognition and executive functions in school-aged children with TGA. METHOD: twenty-one children (14 males, seven females; mean age 7y 4mo; SD 3mo) who underwent neonatal open-heart surgery for TGA using full-flow cardiopulmonary bypass were compared with 21 typically developing age-matched children (12 males, nine females; mean age 7y 6mo; SD 3.8mo) using different neuropsychological measures specifically designed to assess executive function (cognitive and response inhibition, verbal and spatial working memory, and planning). They were also given two ToM tasks (first- and second-order false belief understanding). RESULTS: general IQ was within the normal range in both the TGA group and the comparison group (mean IQ 113 [SD 9.3] and 118 [SD 10.1] respectively), but performance on all executive functions and on ToM (first and second level) was significantly lower in the TGA group (p values of 0.02, 0.01, and 0.004 respectively). A discriminant multivariate analysis provided evidence for cognitive and behavioural inhibition as well as performance on false belief tasks as being the most important contributors to the differentiation between the groups (p=0.03). INTERPRETATION: children with TGA demonstrate great difficulties in exerting cognitive and behavioural inhibition. They also present specific deficits in false belief understanding, which were related to immature executive abilities.

Diagnosis and outcome in congenital ventricular diverticulum and aneurysm.

OBJECTIVE: True congenital ventricular diverticulum and aneurysm in children are very uncommon. We report our experience to clarify the diagnosis and outcome of these little-known entities. METHODS: Twenty-two patients with congenital ventricular outpouchings were identified in our database from 1973 to 2004. Morphologic characteristics (localization, connection to a ventricle, contractility), histologic findings, and cardiac and/or extracardiac abnormalities were analyzed in all 22 patients. Cardiovascular events and clinical courses were reviewed. RESULTS: Congenital ventricular diverticula (n = 16) were characterized by synchronal contractility and three myocardial layers on histologic examination. Two categories of congenital ventricular diverticulum could be identified with regard to their localization: apical and nonapical. Apical diverticula (n = 8) were always associated with midline thoracoabdominal defects and other heart malformations. Nonapical diverticula (n = 8) were always isolated defects. Congenital ventricular aneurysms (n = 6) were characterized by akinesis with paradoxical systolic motion, wide connection to the ventricle, fibrosis on histologic examination that appeared with high signal on T2 weighted magnetic resonance imaging, and absence of other heart or midline thoracoabdominal defects. The outcome was different in these two types of outpouchings: congential ventricular aneurysms were associated with adverse outcomes whereas the prognosis for congenital ventricular diverticula was good. CONCLUSION: Congenital ventricular diverticulum and aneurysm are two distinct entities, with different histologic and morphologic characteristics and outcomes. Assessment of these differential characteristics is of importance for prenatal counseling.