Leif T. Eskedal

First Day of Life Pulse Oximetry Screening to Detect Congenital Heart Defects

OBJECTIVE To evaluate the efficacy of first day of life pulse oximetry screening to detect congenital heart defects (CHDs). STUDY DESIGN We performed a population-based prospective multicenter study of postductal (foot) arterial oxygen saturation (SpO(2)) in apparently healthy newborns after transfer from the delivery suite to the nursery. SpO(2) < 95% led to further diagnostic evaluations. Of 57,959 live births, 50,008 (86%) were screened. In the screened population, 35 CHDs were [corrected] classified as critical (ductus dependent, cyanotic). CHDs were prospectively registered and diagnosed in 658/57,959 (1.1%) [corrected] RESULTS Of the infants screened, 324 (0.6%) failed the test. Of these, 43 (13%) had CHDs (27 critical), and 134 (41%) had pulmonary diseases or other disorders. The remaining 147 infants (45%) were healthy with transitional circulation. The median age for babies with CHDs at failing the test was 6 hours (range, 1-21 hours). For identifying critical CHDs, the pulse oximetry screening had a sensitivity rate of 77.1% (95% CI, 59.4-89.0), specificity rate of 99.4% (95% CI, 99.3-99.5), and a false-positive rate of 0.6% (95% CI, 0.5-0.7). CONCLUSIONS Early pulse oximetry screening promotes early detection of critical CHDs and other potentially severe diseases. The sensitivity rate for detecting critical CHDs is high, and the false-positive rate is low.

Sudden unexpected death in children with congenital heart defects

AIMS Congenital heart defects (CHDs) are the most common birth defects and are an important cause of death in children. The fear of sudden unexpected death has led to restrictions of physical activity and competitive sports. The aim of the present study was to investigate the rate of sudden unexpected deaths unrelated to surgery in children 2-18 years old with CHDs and, secondarily, to determine whether these deaths were related to cardiac disease, comorbidity, or physical activity. METHODS AND RESULTS To identify children with CHDs and to determine the number of deaths, data concerning all 9 43 871 live births in Norway in 1994-2009 were retrieved from the Medical Birth Registry of Norway, the Cardiovascular Disease in Norway project, the Oslo University Hospitals Clinical Registry for Congenital Heart Defects and the Norwegian Cause of Death Registry. Survivors were followed through 2012, and information for the deceased children was retrieved from medical records at Norwegian hospitals. Among 11 272 children with CHDs, we identified 19 (0.2%) children 2-18 years old who experienced sudden unexpected deaths unrelated to cardiac surgery. A cardiac cause of death was identified in seven of these cases. None of the children died during physical activity, whereas two children survived cardiac arrest during sports. CONCLUSION Sudden unexpected death was infrequent among children with CHDs who survived 2 years of age. Comorbidity was common among the children who died. This study indicates that sudden unexpected death in children with CHDs rarely occurs during physical activity.

Motor and Social Development in 6-Month-Old Children with Congenital Heart Defects

OBJECTIVE To assess whether the development of children with varying severity of congenital heart defect (CHD) differs from that of children without CHD at age 6 months. STUDY DESIGN A total of 236 children with CHD were compared with 61 032 children from the Norwegian Mother and Child Cohort Study conducted by the Norwegian Institute of Public Health. Diagnostic and treatment information was retrieved from a nationwide CHD registry. Four groups of CHD were distinguished: mild (n = 92), moderate (n = 50), severe (n = 70), and CHD with comorbidity (n = 24). At child age 6 months, the childrens mothers reported on motor and social development by using the Mother and Child Questionnaire. RESULTS After adjusting for confounders (ie, birth weight), severe CHD increased the odds of gross motor impairment (odds ratio [OR], 3.78; 95% CI, 1.97-7.25) and fine motor impairment (OR, 2.04; 95% CI, 0.96-4.33). CHD with co-morbidity (eg, intestinal malformations) increased the odds of gross motor impairment (OR, 3.00; 95% CI, 0.95-9.51), fine motor impairment (OR, 5.47; 95% CI, 2.03-14.74), and social impairment (OR, 3.43; 95% CI, 1.40-8.41). CONCLUSION Increased odds of motor impairment are present already in infancy in severe CHD and CHD with comorbidity. CHD with comorbidity increases the odds of social impairment.

Emotional reactivity in infants with congenital heart defects: findings from a large case-cohort study in Norway.

Aim:  Advances in medical treatment in recent years have led to dramatically improved survival rates of children with severe congenital heart defects (CHD). However, very little is known about the psychological consequences for these children, particularly during and after the early period of invasive treatment. In this study, we investigated the extent to which the severity of the CHD affects the child’s emotional reactivity at 6 months of age.

Mortality and complications in 3495 children with isolated ventricular septal defects

Background Ventricular septal defects (VSDs) are the most common congenital heart defects (CHDs). Previous studies indicate an increased risk of endocarditis, aortic regurgitation, left ventricular outflow tract obstructions, pulmonary hypertension, arrhythmias and sudden death in patients with isolated VSDs. The present nationwide cohort study reports mortality and cardiac complications requiring hospitalisation or intervention in children with isolated VSDs. Methods and results Medical information concerning all 943 871 live births in Norway in 1994–2009 was retrieved from the Medical Birth Registry of Norway, the Cardiovascular Disease in Norway project, the Oslo University Hospitals Clinical Registry of Congenital Heart Defects and the Norwegian Cause of Death Registry. Isolated VSDs were identified in 3495 children without known chromosomal aberrations or extracardiac malformations. Surgical or catheter-based treatment of VSD was performed in 181 (5.2%) cases. Twelve (0.3%) children with VSDs died before 2013. There was no operative mortality, and no excess mortality in children with isolated VSDs compared with children without VSDs (adjusted HR 0.8 (0.5 to 1.4), p=0.48). The following conditions were recorded as possible cardiac complications of the VSDs: endocarditis in 3 children (0.9‰), aortic regurgitation in 12 children (3.4‰), left ventricular outflow tract obstructions in no children (0.0‰), pulmonary hypertension in 1 child (0.3‰) and arrhythmias in 16 children (4.6‰). Conclusions The entire group of children with isolated VSDs had a favourable prognosis without excess mortality. Cardiac complications requiring hospitalisation or intervention, including endocarditis, aortic regurgitation, left ventricular outflow tract obstructions, pulmonary hypertension and arrhythmias, were infrequent during childhood. Trial registration number NCT02026557.

Adaptation to the birth of a child with a congenital anomaly: a prospective longitudinal study of maternal well-being and psychological distress.

This study explores the stability and change in maternal life satisfaction and psychological distress following the birth of a child with a congenital anomaly using 5 assessments from the Norwegian Mother and Child Cohort Study collected from Pregnancy Week 17 to 36 months postpartum. Participating mothers were divided into those having infants with (a) Down syndrome (DS; n = 114), (b) cleft lip/palate (CLP; n = 179), and (c) no disability (ND; n = 99,122). Responses on the Satisfaction With Life Scale and a short version of the Hopkins Symptom Checklist were analyzed using structural equation modeling, including latent growth curves. Satisfaction and distress levels were highly diverse in the sample, but fairly stable over time (retest correlations: .47-.68). However, the birth of a child with DS was associated with a rapid decrease in maternal life satisfaction and a corresponding increase in psychological distress observed between pregnancy and 6 months postpartum. The unique effects from DS on changes in satisfaction (Cohens d = -.66) and distress (Cohens d = .60) remained stable. Higher distress and lower life satisfaction at later assessments appeared to reflect a persistent burden that was already experienced 6 months after birth. CLP had a temporary impact (Cohens d = .29) on maternal distress at 6 months. However, the overall trajectories did not differ between CLP and ND mothers. In sum, the birth of a child with DS influences maternal psychological distress and life satisfaction throughout the toddler period, whereas a curable condition like CLP has only a minor temporary effect on maternal psychological distress.

Trends in Mortality of Congenital Heart Defects.

OBJECTIVE The aim of the present nationwide cohort study was to describe trends in 1-year mortality in live-born children with congenital heart defects in Norway 1994-2009 and to assess whether changes in the proportion of terminated pregnancies and altered operative mortality have influenced these trends. METHODS Medical information concerning all 954 413 live births, stillbirths, and late-term abortions in Norway, 1994-2009, was retrieved from the Medical Birth Registry of Norway, the Cardiovascular Disease in Norway project, the Oslo University Hospitals Clinical Registry for Congenital Heart Defects and the Norwegian Cause of Death Registry. Survivors were followed through 2012. RESULTS The 1-year cumulative mortality proportion during the study period was 17.4% for children with severe congenital heart defects and 3.0% for children with nonsevere congenital heart defects. The 1-year cumulative mortality proportion among live born children with severe congenital heart defects decreased 3.6% (95% CI: -5.4, -1.5) per year. The total mortality of severe congenital heart defects was unchanged when including stillbirths and late-term abortions with severe congenital heart defects. The proportion of stillbirths or terminated pregnancies with severe congenital heart defects among all pregnancies with severe congenital heart defects, was on average 8.8% over the entire period with an annually increase of 16.6% (11.4, 18.0). The mean operative mortality in children with severe congenital heart defects was 8.4% and decreased by 9.0% (-11.9, -5.9) per year. CONCLUSIONS The 1-year mortality of severe congenital heart defects among live births, 1994-2009, declined in Norway. The downward trend in mortality may be explained by a more frequent use of termination of affected pregnancies, and the reduced operative mortality of severe congenital heart defects.

Longitudinal analysis of emotional problems in children with congenital heart defects: a follow-up from age 6 to 36 months.

Objective: To investigate whether children with varying severity of congenital heart defects (CHDs) have a higher risk of internalizing or externalizing emotional problems at 36 months of age. In addition, to analyze whether a history of emotional problems at 6 or 18 months of age increases the risk of emotional problems at 36 months in children with CHDs. Methods: Prospective data from the Norwegian Mother and Child Cohort Study, conducted by the Norwegian Institute of Public Health, was linked with a nationwide CHD registry, and 175 children with CHDs were identified in a cohort of 44,104 children aged 36 months. Maternal reports on child characteristics were assessed by questionnaires at child age 6, 18, and 36 months. Results: Children with CHDs did not have elevated scores on internalizing or externalizing problems at 36 months of age compared with controls. Not even the children with CHDs with a history of emotional problems at age 6 or 18 months showed an increased risk. Conclusions: The absence of risk of emotional problems at 36 months of age in children with CHDs could be a consequence of the completion of the most extensive medical treatment.

Occurrence and predictors of developmental impairments in 3-year-old children with congenital heart defects.

Objective: To examine the occurrence of developmental impairments in 3-year-old children with varying severity of congenital heart defects (CHD) and to identify predictors associated with developmental impairment in children with severe CHD. Methods: Prospective data collected at birth, 6, 18, and 36 months from the Norwegian Mother and Child Cohort Study (MoBa), conducted by the Norwegian Institute of Public Health, were linked with a nationwide medical CHD registry, and 175 three year olds with CHD in a cohort of 44,044 children were identified. Children with mild/moderate (n = 115) and severe (n = 60) CHD were compared with children without CHD (43,929) on motor, communication, and social impairments as reported by mothers in Norwegian Mother and Child Cohort Study questionnaires. Predictors of developmental impairment were analyzed for the group with severe CHD. Results: Children with severe CHD had >3 times higher odds of communication and gross motor impairments compared with controls, and had 2 times higher odds of any developmental impairment compared with controls. Children with mild and moderate CHD had >2 times higher odds of gross motor impairment but did not otherwise differ from controls. Predictors of impairment identified were previous developmental impairments and smaller head circumference at birth. Conclusion: Children with severe CHD have increased odds of developmental impairments at age 3 years. Early developmental impairments are associated with later developmental impairments, suggesting lasting impairments and not merely temporary delay. Patient-specific conditions at birth should be considered and motor and communication support provided to potentially improve outcomes in children with CHD.

Longitudinal findings from a Norwegian case-cohort study on internalizing problems in children with congenital heart defects

Aim:  To examine the association of the severity of congenital heart defects (CHDs) with internalizing problems in 18‐month‐olds and to explore the extent to which the internalizing problems are influenced by maternal distress and emotional reactivity in the child at age 6 months.