Rabia Liamlahi

Mortality and neurodevelopmental outcome at 1 year of age comparing hybrid and Norwood procedures

OBJECTIVES Neonates with hypoplastic left heart syndrome (HLHS) are at risk of high mortality and neurodevelopmental morbidity. As an alternative to Norwood-type stage I palliation, the hybrid procedure has been developed. It consists of bilateral pulmonary artery banding, catheter-based stenting of the arterial duct and balloon atrioseptostomy and delays open-heart surgery. Thus, it may be associated with a better outcome. The aim of this study was to determine the mortality and neurodevelopmental outcome in patients with HLHS and other univentricular heart (UVH) defects treated with hybrid or Norwood procedures. METHODS Thirty-one children (18 males) with HLHS and other UVH defects undergoing Norwood or hybrid procedure between 2004 and 2008 were consecutively enrolled. Mortality and neurodevelopmental outcome at 1 year of age were determined. RESULTS One-year mortality was 36% (31% in the hybrid vs. 39% in the Norwood group, P=0.71). Predictors of mortality were lower birth weight (P=0.02), older age at first procedure (P=0.02) and smaller size of ascending aorta (P=0.05). Overall, median psychomotor development index (PDI) and mental development index (MDI) of the Bayley Scales of Infant Development II were lower than the norm of 100 [PDI 57 (49-99), P<0.001; MDI 91 (65-109), P=0.002]. No effect of surgical treatment on neurodevelopmental outcome was found. Predictors of impaired motor outcome were length of hospital stay (LOHS) (P=0.01), lower body weight at second procedure (P=0.004) and female sex (P=0.01). Predictors of impaired cognitive outcome were longer mechanical ventilation time (P=0.03), intensive care unit stay (P=0.04) and LOHS (P<0.001), respectively. CONCLUSIONS Mortality at 1 year of age is comparable between patients undergoing hybrid and Norwood procedures. Early neurodevelopmental outcome is significantly impaired in patients with both HLHS and other UVH defects. Multicentre randomized studies are needed to determine the long-term neurodevelopmental outcome of children treated with the hybrid procedure.

Severe Congenital Heart Defects Are Associated with Global Reduction of Neonatal Brain Volumes

OBJECTIVES To determine neonatal global and regional brain volumes in infants with congenital heart disease (CHD) in comparison with healthy controls and to determine brain growth. STUDY DESIGN Prospective cohort study in infants undergoing open-heart surgery for complex CHD. Global and regional volumetric measurements on preoperative cerebral magnetic resonance imaging were manually segmented in children without overt brain lesions. RESULTS Preoperative brain volumetry of 19 patients demonstrates reduction in total and regional brain volumes, without any specific regional predilection compared with 19 healthy control infants (total brain volume reduction: 21%, regional brain volume reduction 8%-28%, all P < .001). CONCLUSIONS Infants with CHD undergoing bypass surgery have smaller brain volumes prior to surgery without a specific regional predilection. This suggests a fetal origin of reduced brain growth.

Gastric emptying after overnight fasting and clear fluid intake: a prospective investigation using serial magnetic resonance imaging in healthy children

BACKGROUND Current guidelines recommend preoperative fasting of 2 h for clear fluids, which is often exceeded in routine clinical practice. Existing data on residual gastric volumes in children do not consider fluid intake within <2 h and rely on the aspiration of gastric contents via a gastric tube. This study evaluated the emptying of clear fluids from the stomach using magnetic resonance imaging (MRI). METHODS Healthy volunteers aged 6-14 years were asked to fast overnight. MRI scans to assess gastric volumes were obtained before and immediately after drinking 7 ml kg(-1) of diluted raspberry syrup and then every 30 min up to 120 min. Volumes were determined by a blinded investigator and indexed gastric fluid/air volumes (GFV(w)/GAV(w)) and half-life (t(1/2)) of GFV(w) course after clear fluid intake were calculated. RESULTS Sixteen children, median age 9.2 (range 6.4-12.8) years, were investigated. Median (range) GFV(w) was 0.62 (0.15-0.97) ml kg(-1) before and 6.68 (4.77-7.78) ml kg(-1) immediately after fluid intake, and 2.92 (0.43-5.04), 1.27 (0.28-3.62), 0.42 (0.07-2.49), and 0.32 (0.04-1.13) ml kg(-1) 30, 60, 90, and 120 min thereafter. Median GFV(w) declined exponentially (t(1/2)=26.1 min). Median individual t(1/2) was 23.6 (range 17.9-47.8) min. GAV(w) showed considerable intra- and inter-individual variation. CONCLUSIONS In healthy school children, gastric emptying after ingestion of clear fluid occurs with a median half-life time of <30 min but with considerable inter-individual variation.

Cerebral lesions on magnetic resonance imaging correlate with preoperative neurological status in neonates undergoing cardiopulmonary bypass surgery

OBJECTIVES To determine the prevalence, spectrum and course of cerebral lesions in neonates with congenital heart disease (CHD) undergoing full flow cardiopulmonary bypass (CPB) surgery using magnetic resonance imaging (MRI) and to examine the correlation between cerebral lesions and clinical neurological abnormalities. METHODS Prospective cohort study of neonates with d-transposition of the great arteries (n = 22), univentricular heart malformation with hypoplastic aortic arch (n = 6) and aortic arch obstructions (n = 2) undergoing CPB. Neonates underwent cerebral MRI and blinded standardized neurological examination before (median day 6) and after surgery (day 13). The MRI findings were compared with those of 20 healthy controls. RESULTS Preoperative cerebral lesions were present in 7 of 30 patients (23%) with isolated mild or moderate white matter injury (WMI) (n = 4), isolated small cerebral stroke (n = 1) and combined WMI and stroke (n = 2). None of the healthy controls had cerebral lesions on MRI. CHD neonates with preoperative cerebral lesions had more neurological abnormalities (P = 0.01) than neonates without cerebral lesions. Low arterial oxygen saturation (P = 0.03) was a risk factor for preoperative cerebral lesions, while balloon atrioseptostomy (P = 0.19) was not. After surgery, preoperative cerebral lesions persisted in 5 of 7 neonates, and 2 neonates (7%) showed signs of additional WMI in their postoperative MRI. CONCLUSIONS In neonates with severe CHD, WMI was the predominant preoperative finding, while cerebral strokes were less frequent. New postoperative lesions were rare. Preoperative neurological abnormalities correlated with the presence of cerebral lesions on MRI.

Residual gastric contents volume does not differ following 4 or 6 h fasting after a light breakfast – a magnetic resonance imaging investigation in healthy non‐anaesthetised school‐age children

While American Society of Anesthesiologists and European Society of Anaesthesiology guidelines recommend 6 h pre‐anaesthetic fasting for food and non‐clear fluids in children, some institutions allow shorter fasting times of 4 h. Aim of this pilot study was to compare weight‐indexed residual gastric contents volumes (GCVw) after 4 vs. 6 h after a light breakfast, using magnetic resonance imaging (MRI) in healthy volunteer children not scheduled for anaesthesia.

Hippocampal volume reduction is associated with intellectual functions in adolescents with congenital heart disease

Background:Adolescents undergoing early cardiopulmonary bypass surgery for congenital heart disease (CHD) may demonstrate a variety of neurocognitive impairments. These impairments can affect overall intellectual functions, but also specific memory deficits, language, and executive functions. As the hippocampus is a critical structure involved in these functions, we sought to determine whether hippocampal volume was reduced in adolescents with CHD and whether altered volumes were related to functional outcome.Methods:At a mean age of 13.8 y, 48 adolescent survivors of childhood cardiopulmonary bypass surgery for CHD and 32 healthy controls underwent neurocognitive testing and cerebral magnetic resonance imaging. Images were quantitatively analyzed using an automated regional segmentation tool (FSL-FIRST).Results:Adolescents with CHD had 10% lower total hippocampal volumes compared with controls. After controlling for total brain volume, total hippocampal volume correlated with total IQ, with working memory, and verbal comprehension in CHD patients, but not in controls.Conclusions:In adolescent survivors of cardiopulmonary bypass surgery for CHD, specific brain regions such as the hippocampus may show long-term persistent alteration and correlate with intellectual deficits, particularly with verbal and memory functions.

Motor dysfunction and behavioural problems frequently coexist with congenital heart disease in school-age children

Patients with congenital heart disease are at risk of neurodevelopmental deficits. Impairments in motor and behavioural function occur frequently, but no information is available concerning the coexistence of deficits in these two developmental domains. This study explored the occurrence of motor and behavioural deficits and their coexistence in children with surgically corrected congenital heart disease.

Persistence of visuo-constructional and executive deficits in adolescents after open-heart surgery

AIM This study assesses whether previously reported performance deficiencies in visuo-constructional and executive functions, using the Rey-Osterrieth Complex Figure Test (ROCFT) in pediatric patients with congenital heart disease (CHD), persist into adolescence. METHODS 53 adolescent CHD patients (mean age 13.7) and 39 healthy controls (mean age 14.1) participated. ROCFT performance was measured by three different scoring methods, focusing either on quantitative (Meyers & Meyers, 1995), qualitative (Wallon & Mesmin, 2009), or both performance aspects (Bernstein & Waber, 1996). Potential confounders (i.e., intelligence and visuomotor integration) and surgery-related risk factors were included in the data analysis. RESULTS Adolescents with CHD demonstrated immature copy and recall approaches on the ROCFT using the qualitative system by Wallon and Mesmin (p<.001). Memory performance was also predicted by Bernstein and Waber scores (p<.03), whereas group differences were not significant according to the other scoring methods. Intelligence and visuomotor skills, but not surgery-related risk factors, were positively correlated with ROCFT performance (each p<.02). Interpretation: Visuoconstructional and executive deficiencies could be found in adolescent patients with CHD. However, not all ROCFT scoring methods were equally apt to detect group differences: especially the qualitative scoring method developed by Wallon and Mesmin seems sufficiently sensitive to detect long-lasting visuo-constructional and executive deficiencies in CHD patients.

Neurodevelopmental profiles of children with congenital heart disease at school age

Objectives To assess 6‐year neurodevelopmental outcomes in a current cohort of children with congenital heart disease (CHD) who underwent cardiopulmonary bypass surgery (CPB), and to determine risk factors for adverse outcomes. Study design Outcomes were examined in 233 prospectively enrolled children with CHD (including 64 with a recognized genetic disorder) who underwent CPB between 2004 and 2009. Follow‐up assessment included standardized neurologic, motor, and cognitive tests. Variables were collected prospectively, and multiple regression analysis was performed to determine independent risk factors for adverse outcome. Results The mean patient age at assessment was 6.3 years (range, 5.1–6.8 years). IQ was lower in children with a genetic disorder (median, 55; range, 17–115) compared with children without a genetic disorder (median, 95; range, 47–135; P < .001). Cognitive and motor performance also were lower in children without a genetic disorder compared with the norm (P < .01 for both). The prevalence of children without a genetic disorder performing below ‐2 SD (IQ 70) was higher than the norm (5.3% vs 2.3%; P = .008), and the prevalence of poor motor performance (<10th percentile) ranged from 21.2% to 41.1% (P < .01 for all). Significant independent risk factors for poor neurodevelopmental outcome included a genetic disorder, longer length of intensive care stay, lower birth weight, postoperative seizures, and lower socioeconomic status. Conclusions Current cohorts of children with CHD undergoing CPB show favorable outcomes but remain at risk for long‐term neurodevelopmental impairments, particularly those with a genetic disorder and a complicated postoperative course. Close neurodevelopmental surveillance is necessary to provide early therapeutic support.

Growth and Intellectual Abilities of Six-Year-Old Children with Congenital Heart Disease

Objective To determine growth and its relationship to IQ in children with congenital heart disease (CHD) undergoing cardiopulmonary bypass surgery within the first year of life. Study design Prospective single‐center cohort study on 143 children (91 males) with different types of CHD (29 univentricular). Children with recognized genetic disorders were excluded. Growth (weight, height, and head circumference [HC]) was assessed at birth, before surgery, and at 1, 4, and 6 years and compared with Swiss growth charts. IQ was assessed at 6 years using standardized tests. Univariate and multivariable linear regressions were performed to determine predictors of HC and IQ at 6 years. Results HC at birth was in the low average range (33rd percentile, P = .03), and weight (49th percentile, P = .23) and length (47th percentile, P = .06) were normal. All growth measures declined until the first surgery, with a catch‐up growth until 6 years for height (44th percentile, P = .07) but not for weight (39th percentile, P = .003) or for HC (23rd percentile, P < .001). Children undergoing univentricular palliation showed poorer height growth than other types of CHD (P = .01). Median IQ at 6 years was 95 (range 50‐135). Lower IQ at 6 years was independently predicted by lower HC at birth, lower socioeconomic status, older age at first bypass surgery, and longer length of intensive care unit stay. Conclusions Smaller HC at birth and postnatal factors are predictive of impaired intellectual abilities at school age. Early identification should alert clinicians to provide early childhood interventions to optimize developmental potential.