Neurodevelopmental Outcome and Health-related Quality of Life in Children With Single-ventricle Heart Disease Before Fontan Procedure

Neurodevelopmental impairment and impaired quality of life constitute a major source of morbidity among children with complex congenital heart disease, in particular for single-ventricle (SV) morphologies. Risk factors and quality of life determining clinical and neurodevelopmental outcome at 2 years of age are examined. In a 2-center cohort study, 48 patients with SV morphology (26 hypoplastic left heart syndrome and 22 other types of univentricular heart defect) have been examined before Fontan procedure between 2010 and 2015. Patients were assessed with the Bayley Scales of Infant and Toddler Development, Third Version (Bayley-III), and the Preschool Children Quality of Life (TAPQOL) questionnaire. A total of 44 patients underwent hybrid procedure (n = 25), Norwood procedure (n = 7), or shunt or banding procedure (n = 12) as first surgery before subsequent bidirectional cavopulmonary anastomosis (n = 48). Median cognitive, language, and motor composite scores on the Bayley-III were 100 (range 65-120), 97 (68-124), and 97 (55-124), respectively. The language composite score was significantly below the norm (P = 0.025). Risk factors for poorer neurodevelopmental outcome were prolonged mechanical ventilation, longer days of hospital stay, and more reinterventions (all P < 0.05). Parents reported a good quality of life for their children. Children undergoing Fontan procedure show a favorable development and good quality of life. More complicated postoperative course and reinterventions constitute risk factors for impaired neurodevelopment. Improving postoperative management and implementing routine follow-up assessments aremeasures to further improve the neurodevelopmental outcome of this high-risk patient population

Reduction of brain volumes after neonatal cardiopulmonary bypass surgery in single ventricle congenital heart disease prior to Fontan completion

BACKGROUND: Little is known about the relationship between brain volumes and neurodevelopmental outcome at two years of age in children with single ventricle congenital heart disease (CHD). We hypothesized that reduced brain volumes may be associated with adverse neurodevelopmental outcome. METHODS: Volumetric segmentation of cerebral MRI scans was done in 44 patients without genetic comorbidities and in eight controls. Neurodevelopmental outcome was assessed with the Bayley-III scales. RESULTS: Gray matter (GM), deep GM, white matter (WM), and cerebrospinal fluid (CSF) volumes were 611±59 ml, 43±4.5 ml, 277±30 ml, and 16.4 ml (IQR 13.1, 23.3 ml). Children undergoing neonatal cardiopulmonary bypass surgery showed smaller deep GM (P=0.005) and WM (P=0.021) volumes. Brain volumes were smaller in patients compared to controls (GM: P=0.017, deep GM: P=0.012, WM: P=0.015), whereas CSF volumes were greater (P=0.014). Of all intracranial volumes, only CSF volume was associated with neurodevelopmental outcome, accounting for 21% (P=0.011) of variability in the cognitive composite score when combined with common risk factors in a multivariable analysis. CONCLUSION: Increased CSF volume represents a significant risk factor for neurodevelopmental impairment in children with single ventricle CHD. Later assessments are warranted to determine the prognostic role of intracranial volumes for long-term outcome.Pediatric Research accepted article preview online, 21 August 2017. doi:10.1038/pr.2017.203