Predictors of behavioural and emotional outcomes in toddlers with congenital heart disease.

OBJECTIVES: Children with congenital heart disease (CHD) are at risk for psychological challenges, including internalising (e.g., depression, anxiety) and externalising (e.g., aggression, inattention) problems. The present study aimed to investigate the development of psychological concerns in early childhood by identifying predictors of behavioural and emotional problems in toddlers with CHD. METHODS: Children with CHD who were seen for neurodevelopmental (ND) evaluation at 12 ± 3 months of age, who completed the Bayley Scales of Infant Development-III (BSID-III) and whose parents completed the Child Behavior Checklist (CBCL), a standardised measure of emotional/behavioural problems at age 24-36 months, were included in the study (n = 144). CBCL scores were compared to test norms and classified as normal or abnormal. A classification tree was used to assess the association between CBCL scores and demographic and clinical variables. RESULTS: Multi-variable tree analyses revealed lower BSID-III language composite scores at age 9-15 months predicted clinical CBCL internalising (p < 0.001), externalising (p = 0.004) and total scores (p < 0.001) at age 24-36 months. Lower maternal education levels also predicted clinical CBCL internalising (p < 0.0001), externalising (p < 0.001) and total scores (p < 0.0001). CONCLUSIONS: Lower language abilities and lower maternal education predict increased behavioural and emotional problems in toddlers with CHD. These risk factors should be considered during routine ND evaluations to allow for earlier identification of children with CHD and their families who may benefit from psychological support.

Accuracy of a portable pulse oximeter in monitoring hypoxemic infants with cyanotic heart disease.

OBJECTIVE: Infants with single ventricle physiology have arterial oxygen saturations between 75 and 85%. Home monitoring with daily pulse oximetry is associated with improved interstage survival. They are typically sent home with expensive, bulky, hospital-grade pulse oximeters. This study evaluates the accuracy of both the currently used Masimo LNCS and a relatively inexpensive, portable, and equipped with Bluetooth technology study device, by comparing with the gold standard co-oximeter. DESIGN: Prospective, observational study. SETTING: Single institution, paediatric cardiac critical care unit, and neonatal ICU. INTERVENTIONS: none. PATIENTS: Twenty-four infants under 12 months of age with baseline oxygen saturation less than 90% due to cyanotic CHD. MEASUREMENTS AND RESULTS: Pulse oximetry with WristOx2 3150 with infant sensors 8008 J (study device) and Masimo LCNS saturation sensor connected to a Philips monitor (hospital device) were measured simultaneously and compared to arterial oxy-haemoglobin saturation measured by co-oximetry. Statistical analysis evaluated the performances of each and compared to co-oximetry with Schuirmann's TOST equivalence tests, with equivalence defined as an absolute difference of 5% saturation or less. Neither the study nor the hospital device met the predefined standard for equivalence when compared with co-oximetry. The study device reading was on average 4.0% higher than the co-oximeter, failing to show statistical equivalence (p = 0.16). The hospital device was 7.4% higher than the co-oximeter and also did not meet the predefined standard for equivalence (p = 0.97). CONCLUSION: Both devices tended to overestimate oxygen saturation in this patient population when compared to the gold standard, co-oximetry. The study device is at least as accurate as the hospital device and offers the advantage of being more portable with Bluetooth technology that allows reliable, efficient data transmission. Currently FDA-approved, smaller portable pulse oximeters can be considered for use in home monitoring programmes.

Preschool Neurodevelopmental Outcomes in Children with Congenital Heart Disease.

OBJECTIVE: To describe preschool neurodevelopmental outcomes of children with complex congenital heart disease (CHD), who were evaluated as part of a longitudinal cardiac neurodevelopmental follow-up program, as recommended by the American Heart Association and the American Academy of Pediatrics, and identify predictors of neurodevelopmental outcomes in these children. STUDY DESIGN: Children with CHD meeting the American Heart Association/American Academy of Pediatrics high-risk criteria for neurodevelopmental delay were evaluated at 4-5 years of age. Testing included standardized neuropsychological measures. Parents completed measures of child functioning. Scores were compared by group (single ventricle [1V]; 2 ventricles [2V]; CHD plus known genetic condition) to test norms and classified as: normal (within 1 SD of mean); at risk (1-2 SD from mean); and impaired (>2 SD from mean). RESULTS: Data on 102 patients were analyzed. Neurodevelopmental scores did not differ based on cardiac anatomy (1V vs 2V); both groups scored lower than norms on fine motor and adaptive behavior skills, but were within 1 SD of norms. Patients with genetic conditions scored significantly worse than 1V and 2V groups and test norms on most measures. CONCLUSIONS: Children with CHD and genetic conditions are at greatest neurodevelopmental risk. Deficits in children with CHD without genetic conditions were mild and may not be detected without formal longitudinal testing. Parents and providers need additional education regarding the importance of developmental follow-up for children with CHD.

Neurodevelopmental outcomes at 2 and 4 years in children with congenital heart disease.

BACKGROUND AND OBJECTIVES: Children with congenital heart disease (CHD) are at risk for neurodevelopmental (ND) delays. The purpose of this study is to compare the ND testing results of children with CHD at 2 and 4 years of age and determine if rates of ND delays change over time. METHODS: Children with CHD completed the Bayley Scales of Infant Development-III (BSID-III) at 2 years of age, and standardized neuropsychological measures at 4 years. Scores were compared with test norms and were classified as: average (within one SD of test mean); at risk (1-2 SDs from the test mean); and delayed (>2 SD from test mean). Pearson correlations and McNemar's exact tests were performed to determine the relationship between test scores at the two times of assessment. RESULTS: Sixty-four patients completed evaluations at 24 ± 3 months of age and 4 years of age. BSID-III cognitive and fine motor scores were correlated with preschool IQ and fine motor scores, r = .75 to .87, P < .0001. Agreement in score categories was 79% for cognitive and 61% for fine motor. More patients had at risk or delayed scores at age 4 vs age 2 (P ≤ .01). CONCLUSION(S): Despite significant correlations between 2- and 4-year-old test scores, many patients who scored in the average range at age 2 showed deficits at age 4. BSID-III scores at age 2 may underestimate delays. Therefore, longitudinal ND assessment is recommended.

Growth Restriction in Infants and Young Children with Congenital Heart Disease.

OBJECTIVE: The purpose of this study was to determine the prevalence of growth restriction in infants and young children with congenital heart disease (CHD) and investigate the relationship between poor growth, feeding difficulties, cardiac classification, and nutrition intervention on outcomes. DESIGN: This is a prospective observational cohort study of infants and young children with CHD aged 0-3 years admitted to hospital for cardiac surgery. Anthropometry, growth history, cardiac classification, cardiac diagnosis, feeding difficulty, and nutrition intervention data were collected for 78 participants. RESULTS: Many participants demonstrated growth restriction as evidenced by a z-score ≤ -2 for population growth parameters including weight/age z-score (n = 18, 23%), height/age z-score (n = 16, 21%), and weight/height z-score (n = 12, 18%). Increased hospital length of stay was associated with factors including faltering growth preadmission (P = .009), tube feeding required preadmission (P = .002), diagnosis of cyanotic CHD (P = .015), and presence of a feeding difficulty (P = .015). CONCLUSIONS: Growth restriction remains an ongoing problem in children with CHD. Faltering growth preadmission and lower growth parameters were associated with an increased hospital length of stay. Nutritional screening from diagnosis may detect growth faltering, improve access to early nutrition intervention, and improve patient outcomes.

Impact of educational video on critical congenital heart disease screening.

OBJECTIVE: To assess the status of pulse oximetry screening and barriers to implementing screening programs. METHODS: This was a prospective pre-post intervention survey of nurse managers and medical directors of hospital-based birthing centers in Oregon, Idaho, and Southern Washington. The intervention was a 7-minute video demonstrating and discussing pulse oximetry screening for critical congenital heart disease. RESULTS: Analysis of matched pairs showed a significant increase in the use of pulse oximetry screening during the study period from 52% to 73% (P < .0001). Following implementation of the video, the perception of all queried potential barriers decreased significantly among individuals from hospitals self-identified as nonscreening at baseline. Viewing the educational video was associated with an increase in the percentage of individuals from nonscreening hospitals that rated screening as "very beneficial" (45% vs 90%, P = .0001). CONCLUSIONS: An educational video was associated with improved opinions of pulse oximetry screening among hospitals not currently screening.

Accuracy of pulse oximetry in children.

OBJECTIVE: For children with cyanotic congenital heart disease or acute hypoxemic respiratory failure, providers frequently make decisions based on pulse oximetry, in the absence of an arterial blood gas. The study objective was to measure the accuracy of pulse oximetry in the saturations from pulse oximetry (SpO2) range of 65% to 97%. METHODS: This institutional review board-approved prospective, multicenter observational study in 5 PICUs included 225 mechanically ventilated children with an arterial catheter. With each arterial blood gas sample, SpO2 from pulse oximetry and arterial oxygen saturations from CO-oximetry (SaO2) were simultaneously obtained if the SpO2 was ≤ 97%. RESULTS: The lowest SpO2 obtained in the study was 65%. In the range of SpO2 65% to 97%, 1980 simultaneous values for SpO2 and SaO2 were obtained. The bias (SpO2 - SaO2) varied through the range of SpO2 values. The bias was greatest in the SpO2 range 81% to 85% (336 samples, median 6%, mean 6.6%, accuracy root mean squared 9.1%). SpO2 measurements were close to SaO2 in the SpO2 range 91% to 97% (901 samples, median 1%, mean 1.5%, accuracy root mean squared 4.2%). CONCLUSIONS: Previous studies on pulse oximeter accuracy in children present a single number for bias. This study identified that the accuracy of pulse oximetry varies significantly as a function of the SpO2 range. Saturations measured by pulse oximetry on average overestimate SaO2 from CO-oximetry in the SpO2 range of 76% to 90%. Better pulse oximetry algorithms are needed for accurate assessment of children with saturations in the hypoxemic range.

Results from the New Jersey statewide critical congenital heart defects screening program.

BACKGROUND AND OBJECTIVE: New Jersey was the first state to implement legislatively mandated newborn pulse oximetry screening (POxS) in all licensed birthing facilities to detect critical congenital heart defects (CCHDs). The objective of this report was to evaluate implementation of New Jersey's statewide POxS mandate. METHODS: A 2-pronged approach was used to collect data on infants screened in all New Jersey birthing facilities from August 31, 2011, through May 31, 2012. Aggregate screening results were submitted by each birthing facility. Data on failed screens and clinical characteristics of those newborns were reported to the New Jersey Birth Defects Registry (NJBDR). Three indicators were used to distinguish the added value of mandated POxS from standard clinical care: prenatal congenital heart defect diagnosis, cardiology consultation or echocardiogram indicated or performed before PoxS, or clinical findings at the time of POxS warranting a pulse oximetry measurement. RESULTS: Of 75,324 live births in licensed New Jersey birthing facilities, 73,320 were eligible for screening, of which 99% were screened. Forty-nine infants with failed POxS were reported to the NJBDR, 30 of whom had diagnostic evaluations solely attributable to the mandated screening. Three of the 30 infants had previously unsuspected CCHDs and 17 had other diagnoses or non-CCHD echocardiogram findings. CONCLUSIONS: In the first 9 months after implementation, New Jersey achieved a high statewide screening rate and established surveillance mechanisms to evaluate the unique contribution of POxS. The screening mandate identified 3 infants with previously unsuspected CCHDs that otherwise might have resulted in significant morbidity and mortality and also identified other significant secondary targets such as sepsis and pneumonia.

Temporal trends in survival among infants with critical congenital heart defects.

OBJECTIVE: To evaluate the trends in survival for infants with critical congenital heart defects (CCHDs) and to examine the potential impact of timing of diagnosis and other prognostic factors on survival. METHODS: We performed a retrospective population-based cohort study in infants born with structural congenital heart defects (CHDs) between 1979 and 2005 and ascertained by the Metropolitan Atlanta Congenital Defects Program. We estimated Kaplan-Meier survival probabilities for 12 CCHD phenotypes by birth era and timing of diagnosis among infants without noncardiac defects or chromosomal disorders and used stratified Cox proportional hazards models to assess potential prognostic factors. RESULTS: Of 1 056 541 births, there were 6965 infants with CHDs (1830 with CCHDs). One-year survival was 75.2% for those with CCHDs (n = 1336) vs 97.1% for those with noncritical CHDs (n = 3530; P < .001). One-year survival for infants with CCHDs improved from 67.4% for the 1979-1993 birth era to 82.5% for the 1994-2005 era (P < .001). One-year survival was 71.7% for infants with CCHDs diagnosed at ≤1 day of age (n = 890) vs 82.5% for those with CCHDs diagnosed at >1 day of age (n = 405; P < .001). There was a significantly higher risk of 1-year mortality for infants with an earlier birth era, earlier diagnosis, and low birth weight and whose mothers were <30 years old. CONCLUSIONS: One-year survival for infants with CCHDs has been improving over time, yet mortality remains high. Later diagnosis is associated with improved 1-year survival. These benchmark data and identified prognostic factors may aid future evaluations of the impact of pulse oximetry screening on survival from CCHDs.