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Children with hypoplastic left heart syndrome are at a risk for neurodevelopmental delays. Current guidelines recommend systematic evaluation and management of neurodevelopmental outcomes with referral for early intervention services. The Single Ventricle Reconstruction Trial represents the largest cohort of children with hypoplastic left heart syndrome ever assembled. Data on life events and resource utilisation have been collected annually. We sought to determine the type and prevalence of early intervention services used from age 1 to 4 years and factors associated with utilisation of services.
Data from 14-month neurodevelopmental assessment and annual medical history forms were used. We assessed the impact of social risk and geographic differences. Fisher exact tests and logistic regression were used to evaluate associations.
Annual medical history forms were available for 302 of 314 children. Greater than half of the children (52–69%) were not receiving services at any age assessed, whereas 20–32% were receiving two or more therapies each year. Utilisation was significantly lower in year 4 (31%) compared with years 1–3 (with a range from 40 to 48%) (p<0.001). Social risk factors were not associated with the use of services at any age but there were significant geographic differences. Significant delay was reported by parents in 18–43% of children at ages 3 and 4.
Despite significant neurodevelopmental delays, early intervention service utilisation was low in this cohort. As survival has improved for children with hypoplastic left heart syndrome, attention must shift to strategies to optimise developmental outcomes, including enrolment in early intervention when merited.
To assess foetal electrocardiographic intervals across gestational age among foetuses with and without congenital heart disease, and to investigate differences between groups.
A prospective observational cohort study.
Center for Prenatal Pediatrics, Morgan Stanley Children’s Hospital of NewYork-Presbyterian.
Population or sample
A total of 92 participants with singleton pregnancies, 41 with normal anatomy and 51 with congenital heart disease were included in this study.
Using a maternal abdominal monitor, foetal electrocardiogram was obtained serially from foetuses with and without congenital heart disease at 20–24 weeks (F1), 28–32 weeks (F2), and 34–38 weeks (F3) of gestation. A signal-averaged waveform was calculated, and PR, QRS, and QT intervals were measured. Intervals from controls were compared with gestational age norms. Using Pearson’s correlation coefficient, we analysed the relationship between gestational age and foetal electrocardiographic intervals. Intervals from control and congenital heart disease foetuses were compared by Student’s t-test.
PR (r=0.333, p=0.02) and QRS (r=0.248, p=0.05) intervals correlated with gestational age only among controls. QRS intervals in foetuses with congenital heart disease were significantly longer than controls at F1 (63±6 versus 52±5 ms, p<0.001), F2 (61±8 versus 56±7 ms, p=0.02), and F3 (64±10 versus 56±9 ms, p=0.007).
PR and QRS intervals lengthen across gestational age among foetuses with normal cardiac anatomy but not in foetuses with congenital heart diseases. As early as 20 weeks of gestation, differences between foetuses with and without congenital heart disease are discernible, with congenital heart disease foetuses demonstrating longer QRS intervals compared with controls.
Despite improvements in outcomes after completion of the Fontan circulation, long-term functional state varies. We sought to identify pre- and postoperative characteristics associated with overall function.
Methods and Results
We analyzed data from 476 survivors with the Fontan circulation enrolled in the Pediatric Heart Network Fontan Cross-sectional Study. Mean age at creation of the Fontan circulation was 3.4 plus or minus 2.1 years, with a range from 0.7 to 17.5 years, and time since completion was 8.7 plus or minus 3.4 years, the range being from 1.1 to 17.3 years. We calculated a functional score for the survivors by averaging the percentile ranks of ventricular ejection fraction, maximal consumption of oxygen, the physical summary score for the Child Health Questionnaire, and a function of brain natriuretic peptide. The mean calculated score was 49.5 plus or minus 17.3, with a range from 3 to 87. After adjustment for time since completion of the circulation, we found that a lower score, and hence worse functional state, was associated with: right ventricular morphology (p less than 0.001), higher ventricular end-diastolic pressure (p equals 0.003) and lower saturations of oxygen (p equals 0.047) prior to completion of the Fontan circulation, lower income for the caregiver (p equals 0.003), and, in subjects without a prior superior cavopulmonary anastomosis, arrhythmias after completion of the circulation (p equals 0.003). The model explained almost one-fifth (18%) of the variation in the calculated scores. The score was not associated with surgical centre, sex, age, weight, fenestration, or the period of stay in hospital after completion of the Fontan circuit. A validation model, using 71 subjects randomly excluded from initial analysis, weakly correlated (R equals 0.17, p equals 0.16) with the score calculated from the dataset.
Right ventricular morphology, higher ventricular end-diastolic pressure and lower saturations of oxygen prior to completion of the Fontan circuit, lower income for the provider of care, and arrhythmias after creation of the circuit, are all associated with a worse functional state. Unmeasured factors also influence outcomes.
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