ABSTRACT
Objectives: Neonates with single ventricle congenital heart disease are at risk for structural cerebral abnormalities. Little is known about the further evolution of cerebral abnormalities until Fontan procedure. Methods: Between August 2012 and July 2015, we conducted a prospective cross-sectional two centre study using cerebral magnetic resonance imaging (MRI) and neuro-developmental outcome assessed by the Bayley-III. Forty-seven children (31 male) were evaluated at a mean age of 25.9 ± 3.4 months with hypoplastic left heart syndrome (25) or other single ventricle (22). Results: Cerebral MRI was abnormal in 17 patients (36.2%) including liquor space enlargements (10), small grey (9) and minimal white (5) matter injuries. Eight of 17 individuals had combined lesions. Median (range) cognitive composite score (CCS) (100, 65-120) and motor composite score (MCS) (97, 55-124) were comparable to the reference data, while language composite score (LCS) (97, 68-124) was significantly lower ( P = 0.040). Liquor space enlargement was associated with poorer performance on all Bayley-III subscores (CCS: P = 0.02; LCS: P = 0.002; MCS: P = 0.013). The number of re-operations [odds ratio (OR) 2.2, 95% confidence interval (CI) 1.1-4.3] ( P = 0.03) and re-interventions (OR 2.1, 95% CI 1.1-3.8) ( P = 0.03) was associated with a higher rate of overall MRI abnormalities. Conclusions: Cerebral MRI abnormalities occur in more than one third of children with single ventricle, while the neuro-developmental status is less severely affected before Fontan procedure. Liquor space enlargement is the predominant MRI finding associated with poorer neuro-developmental status, warranting further studies to determine aetiology and further evolution until school-age.
Subject(s)
Abnormalities, Multiple/diagnostic imaging , Brain/abnormalities , Heart Defects, Congenital/diagnosis , Neurodevelopmental Disorders/etiology , Abnormalities, Multiple/psychology , Brain/diagnostic imaging , Child, Preschool , Cross-Sectional Studies , Female , Fontan Procedure , Heart Defects, Congenital/psychology , Heart Defects, Congenital/surgery , Heart Ventricles/abnormalities , Humans , Hypoplastic Left Heart Syndrome/diagnosis , Hypoplastic Left Heart Syndrome/psychology , Hypoplastic Left Heart Syndrome/surgery , Infant , Magnetic Resonance Imaging , Male , Neuroimaging/methods , Neuropsychological TestsABSTRACT
A 22-year-old woman presented to the emergency room of a local hospital with pleuritic chest pain. She regularly worked out and admitted to taking performance-enhancing drugs (PEDs). Clinical findings and further diagnostic work up revealed a diagnosis of perimyocarditis, and adequate therapy was initiated. During the course of the first day, the patient had to be intubated and mechanically ventilated. A diagnosis of bilateral pneumonia and acute respiratory distress syndrome (ARDS) due to an infection by rhinovirus spp was made. A smoking habit, the intense physical training and the use of PED's may have exacerbated the course of the viral pneumonia. After 12â days the patient could be extubated. The length of stay in the intensive care unit was 16â days. After hospital discharge, the patient went to a pulmonary rehabilitation facility for 2â weeks. The outcome was favourable and the patient resumed her strength and endurance training.