Erica Sood

Early Developmental Outcome in Children With Hypoplastic Left Heart Syndrome and Related Anomalies The Single Ventricle Reconstruction Trial

Background— Survivors of the Norwood procedure may experience neurodevelopmental impairment. Clinical trials to improve outcomes have focused primarily on methods of vital organ support during cardiopulmonary bypass. Methods and Results— In the Single Ventricle Reconstruction trial of the Norwood procedure with modified Blalock-Taussig shunt versus right-ventricle-to-pulmonary-artery shunt, 14-month neurodevelopmental outcome was assessed by use of the Psychomotor Development Index (PDI) and Mental Development Index (MDI) of the Bayley Scales of Infant Development-II. We used multivariable regression to identify risk factors for adverse outcome. Among 373 transplant-free survivors, 321 (86%) returned at age 14.3±1.1 (mean±SD) months. Mean PDI (74±19) and MDI (89±18) scores were lower than normative means (each P<0.001). Neither PDI nor MDI score was associated with type of Norwood shunt. Independent predictors of lower PDI score (R2=26%) were clinical center (P=0.003), birth weight <2.5 kg (P=0.023), longer Norwood hospitalization (P<0.001), and more complications between Norwood procedure discharge and age 12 months (P<0.001). Independent risk factors for lower MDI score (R2=34%) included center (P<0.001), birth weight <2.5 kg (P=0.04), genetic syndrome/anomalies (P=0.04), lower maternal education (P=0.04), longer mechanical ventilation after the Norwood procedure (P<0.001), and more complications after Norwood discharge to age 12 months (P<0.001). We found no significant relationship of PDI or MDI score to perfusion type, other aspects of vital organ support (eg, hematocrit, pH strategy), or cardiac anatomy. Conclusions— Neurodevelopmental impairment in Norwood survivors is more highly associated with innate patient factors and overall morbidity in the first year than with intraoperative management strategies. Improved outcomes are likely to require interventions that occur outside the operating room. Clinical Trial Registration— URL: http://www.clinicaltrials.gov. Unique identifier: NCT00115934.

Factors associated with neurodevelopment for children with single ventricle lesions.

OBJECTIVE To measure neurodevelopment at 3 years of age in children with single right-ventricle anomalies and to assess its relationship to Norwood shunt type, neurodevelopment at 14 months of age, and patient and medical factors. STUDY DESIGN All subjects in the Single Ventricle Reconstruction Trial who were alive without cardiac transplant were eligible for inclusion. The Ages and Stages Questionnaire (ASQ, n = 203) and other measures of behavior and quality of life were completed at age 3 years. Medical history, including measures of growth, feeding, and complications, was assessed through annual review of the records and phone interviews. The Bayley Scales of Infant Development, Second Edition (BSID-II) scores from age 14 months were also evaluated as predictors. RESULTS Scores on each ASQ domain were significantly lower than normal (P < .001). ASQ domain scores at 3 years of age varied nonlinearly with 14-month BSID-II. More complications, abnormal growth, and evidence of feeding, vision, or hearing problems were independently associated with lower ASQ scores, although models explained <30% of variation. Type of shunt was not associated with any ASQ domain score or with behavior or quality-of-life measures. CONCLUSION Children with single right-ventricle anomalies have impaired neurodevelopment at 3 years of age. Lower ASQ scores are associated with medical morbidity, and lower BSID-II scores but not with shunt type. Because only a modest percentage of variation in 3-year neurodevelopmental outcome could be predicted from early measures, however, all children with single right-ventricle anomalies should be followed longitudinally to improve recognition of delays.

Predictive value of perioperative near-infrared spectroscopy for neurodevelopmental outcomes after cardiac surgery in infancy

OBJECTIVE The study objective was to expand on prior research examining intraoperative regional cerebral oxygen saturation (rSO(2)) measured by near-infrared spectroscopy (NIRS) by evaluating the predictive value of perioperative NIRS monitoring for neurodevelopmental outcomes after infant cardiac surgery. METHODS Cross-sectional neurodevelopmental evaluation at 24 months of age with the Bayley Scales of Infant and Toddler Development, Third Edition was performed for patients who underwent cardiac surgery with perioperative NIRS monitoring between 2007 and 2010. Retrospective clinical data were extracted from the electronic medical record. Evaluation of selected NIRS measures, including preoperative rSO(2) (baseline) as well as rSO(2) nadir and percent decrease from baseline during the intraoperative and early postoperative periods, was undertaken. RESULTS Perioperative NIRS and neurodevelopmental data were available for 31 patients without chromosomal anomalies who underwent cardiac surgery during infancy at a median age of 0.43 months. Optimal thresholds on NIRS measures identified through receiver operating characteristic analyses were intraoperative percent decrease of 52% for receptive communication delay and postoperative rSO(2) nadir of 56% for cognitive delay and 49% for gross motor delay. When considered in conjunction with other clinical characteristics in stepwise linear regression analyses, intraoperative percent decrease of more than 52% entered into the final model for receptive communication outcome and postoperative rSO(2) nadir of less than 56% entered into the final model for cognitive outcome. CONCLUSIONS Perioperative NIRS monitoring seems to enhance the ability to predict neurodevelopmental outcome. Specific NIRS measures associated with neurodevelopmental outcome, as well as optimal thresholds, seem to differ across the continuum of the perioperative period.

Predictive value of near-infrared spectroscopy on neurodevelopmental outcome after surgery for congenital heart disease in infancy

OBJECTIVE To explore the relationship between intraoperative regional cerebral oxygen saturation (rSO(2)) measured by near-infrared spectroscopy (NIRS) and neurodevelopmental outcome in children after cardiac surgery. METHODS Cross-sectional neurodevelopmental evaluation at 2 years of age was performed in a cohort of young infants who had surgery for congenital heart disease in 2007. The third edition of Bayley Scales of Infant and Toddler Development (Bayley-III) was used to assess cognitive, language, and motor functioning. Clinical and perioperative data were collected, including intraoperative rSO(2) nadir, rSO(2) percent decrease from baseline, and cumulative minutes of at least 20%, 30%, and 40% decrease from baseline. RESULTS Twenty-seven patients without chromosomal abnormality were included in analyses. Mean Bayley-III scores fell within 1 standard deviation of the normative mean. Stepwise regression analyses of patient- and procedure-related variables, including rSO(2), demonstrated that cognitive ability was predicted by length of hospital stay and premature birth (58.1% of variance), receptive communication was predicted by length of hospital stay and rSO(2) nadir (40.2% of variance), expressive communication was predicted by birth weight (26.2% of variance), fine motor functioning was predicted by duration of cardiac intensive care unit stay (41.4% of variance), and gross motor functioning was predicted by the presence of a significant comorbidity (43.5% of variance). CONCLUSIONS In a contemporary cohort of infants undergoing surgery for congenital heart disease, neurodevelopmental outcomes at 2 years of age are largely influenced by patient-related characteristics. Although receptive communication appears to be influenced by rSO(2) nadir, the predictive value of NIRS remains unclear.

Mother-Father Informant Discrepancies Regarding Diabetes Management: Associations With Diabetes-Specific Family Conflict and Glycemic Control

OBJECTIVE To examine the relationship of mother-father informant discrepancies regarding diabetes management to diabetes-specific family conflict and glycemic control. METHODS One hundred thirty-six mothers and fathers of youth with Type 1 diabetes reported on the youths diabetes management, diabetes-specific family conflict, and amount of paternal involvement in diabetes care. Glycosylated hemoglobin A1c (HbA1c) was used to measure glycemic control. RESULTS As hypothesized, mother-father discrepancies regarding diabetes management were positively associated with frequency of diabetes-specific family conflict. Contrary to hypotheses, mother-father discrepancies regarding diabetes management predicted poorer glycemic control for youth with less involved fathers only. CONCLUSIONS Results highlight the importance of caregivers being consistent about pediatric illness management and support the idea that informant discrepancies represent an important window into the functioning of the family system.

Developmental Care in North American Pediatric Cardiac Intensive Care Units: Survey of Current Practices.

Background:Developmental care practices across pediatric cardiac intensive care units (CICUs) have not previously been described. Purpose:To characterize current developmental care practices in North American CICUs. Methods:A 47-item online survey of developmental care practices was developed and sent to 35 dedicated pediatric CICUs. Staff members who were knowledgeable about developmental care practices in the CICU completed the survey. Findings/Results:Completed surveys were received from 28 CICUs (80% response rate). Eighty-nine percent reported targeted efforts to promote developmental care, but only 50% and 43% reported having a developmental care committee and holding developmental rounds, respectively. Many CICUs provide darkness for sleep (86%) and indirect lighting for alertness (71%), but fewer provide low levels of sound (43%), television restrictions (43%), or designated quiet times (21%). Attempts to cluster care (82%) and support self-soothing during difficult procedures (86%) were commonly reported, but parental involvement in these activities is not consistently encouraged. All CICUs engage in infant holding, but practices vary on the basis of medical status and only 46% have formal holding policies. Implications for Practice:Implementation of developmental care in the CICU requires a well-planned process to ensure successful adoption of practice changes, beginning with a strong commitment from leadership and a focus on staff education, family support, value of parents as the primary caregivers, and policies to increase consistency of practice. Implications for Research:Future studies should examine the short- and long-term effects of developmental care practices on infants born with congenital heart disease and cared for in a pediatric CICU.

Supporting Development in Children With Congenital Heart Disease

Children with congenital heart disease (CHD) are exceptionally resilient. After extensive surgeries and hospitalizations, they typically go on to live full, meaningful lives. However, some children with CHD experience developmental and learning differences and benefit from extra help to succeed in school, social relationships, and future employment.1 CHD can result in changes to blood flow to the brain before and after birth, and this might affect brain development. Studies have found that the brains of children with some forms of CHD are less mature at birth. Children who have long hospital stays or other complications (premature birth or genetic/neurologic conditions) are also at risk. Studies are underway to …

Developmental screening in children with CHD: Ages and Stages Questionnaires

OBJECTIVE Standardised developmental screening tools are important for the evaluation and management of developmental disorders in children with CHD; however, psychometric properties and clinical utility of screening tools, such as the Ages & Stages Questionnaires, Third Edition (ASQ-3), have not been examined in the CHD population. We hypothesised that the ASQ-3 would be clinically useful for this population. Study design ASQ-3 developmental classifications for 163 children with CHD at 6, 12, 24, and/or 36 months of age were compared with those obtained from concurrent developmental testing with the Bayley Scales of Infant and Toddler Development, Third Edition. RESULTS When ASQ-3 screening failure was defined as ⩾1 SD below the normative mean, specificity (⩾81.9%) and negative predictive value (⩾81.0%) were high across ASQ-3 areas. Sensitivity was high for gross motor skills (79.6%), increased with age for communication (35.7-100%), and generally decreased with age for problem solving (73.1-50.0%). When ASQ-3 screening failure was defined as ⩾2 SD below the normative mean, specificity (⩾93.6%) and positive predictive value (⩾74.5%) were generally high across ASQ-3 areas, but sensitivity was low (31.1%) to fair (62.8%). The ASQ-3 showed improved accuracy in predicting delays over clinical risk factors alone. CONCLUSIONS The ASQ-3 appears to be a clinically useful tool for screening development in children with CHD, although its utility varied on the basis of developmental area and time point. Clinicians are encouraged to refer children scoring ⩾1 SD below the normative mean on any ASQ-3 area for formal developmental evaluation.

Childhood Psychosocial Determinants of Cardiovascular Health

Understanding risk and protective factors that impact cardiovascular health is of utmost importance. There is ample evidence that cardiovascular health begins in childhood, tracks over time, and is subject to adverse social influences. This paper reviews key studies examining the relations of psychosocial factors in childhood to cardiovascular health in adulthood. The existing literature provides evidence for both individual and cumulative effects of childhood psychosocial factors on adult cardiovascular health across the population, although the specific mechanisms underlying these relationships are not yet fully understood. This paper also includes a discussion of evidence-based strategies for prevention and treatment of childhood psychosocial problems. The extent to which these programs lead to improved cardiovascular health in high-risk groups or across the population by impacting psychosocial factors has not yet been studied, but is a clear future direction for research and policy.

Preventing Cardiovascular Disease Going Beyond Conventional Risk Assessment

The rise of cardiovascular disease (CVD) as a leading cause of medical morbidity and mortality worldwide has long been thought to be a consequence of industrialization.1 As industrialization has spread globally, CVD has risen as a worldwide cause of illness and death. Diets higher in saturated fat and salt, sedentary lifestyle, tobacco use, and obesity, known cardiovascular risk factors, all appear to accompany industrialization. However, it is unlikely that a “return to nature” will solve the CVD problem. A positive consequence of industrialization is a longer life expectancy in part as a result of a more stable food supply, modern control of infectious diseases, and reduced perinatal morbidity. Because CVD has a long incubation period, perhaps the most potent cardiovascular risk factor is age. One “cause” of higher disease rates may be simply living long enough to acquire heart disease. Article see p 245 If the oversimplifications above told the entire story, CVD rates would affect the population equally across all social strata and in all countries. However, this is not the case. After age, the second most ignored cardiovascular risk factor is socioeconomic status, with those at the lower end of the spectrum having dramatically higher rates of CVD.2 Although the upper classes might have been the first to experience the rise in CVD rates early in the course of industrialization, the lower classes have suffered disproportionately as industrialization progressed.1 In analyses structured to emphasize the impact of socioeconomic status on risk, the gradient from the lowest to the highest classes may be as much …