Children with FASD-Evolving Patterns of Developmental Problems and Intervention Costs in Ages 0 through 16 in Finland.

This is a retrospective chart review of 55 persons (mean age 11 years, range 2-28 years) diagnosed with fetal alcohol spectrum disorder (FASD) in one Finnish central hospital. The aim was to determine typical problems and interventions and estimate their costs during different periods of childhood between ages 0 and 16. During the first year, 29/38 (76.3%) were treated in the neonatal intensive care unit, 29/43 (67.4%) received physiotherapy, 15/43 (34.9%) were diagnosed with congenital malformation, 8/43 (18.6%) had heart defects. Between 1 and 6 years, 39/45 (86.7%) received occupational therapy, 25/45 (55.6%) speech therapy, and 12/45 (26.7%) were diagnosed with strabismus. Between 7 and 12 years, 25/37 (67.6%) were diagnosed with ADHD and special education was recommended for 30/37 (81.1%). Learning disorders and the need for psychiatric care increased with age. Between 13 and 16 years, 12/15 (80%) were treated in the psychiatric unit, and by this age, 8/15 (53.3%) were diagnosed with intellectual disability. Before 16 years, 44/55 (80%) were placed out of home, which caused 78.5% of the estimated cumulative mean extra costs of EUR 1,077,000 in 2022 currency. Except for psychiatric costs, health care costs were highest during early years. Charting typical patterns of problems may help in identifying children with FASD and planning follow-ups, content of assessments, and interventions.

Changes in Motor Development During a 4-Year Follow-up on Children With Univentricular Heart Defects.

PURPOSE: To compare changes in motor development from 1 to 5 years of age among 18 children with hypoplastic left heart syndrome and 12 with univentricular heart to 42 children without heart defect. METHODS: Motor development was assessed with the Alberta Infant Motor Scale and Movement Assessment Battery for Children (Movement ABC). RESULTS: Children with hypoplastic left heart syndrome or univentricular heart had significantly lower scores on the Alberta Infant Motor Scale test at the age of 1 and on the Movement ABC test at the age of 5 years compared with controls. Children with clear abnormalities on brain magnetic resonance imaging had lower scores compared with those with normal images or mild changes, and their relative motor scores decreased during follow-up. CONCLUSIONS: Some children with univentricular heart defects may benefit from physiotherapeutic interventions to support their motor development.

Motor development of infants with univentricular heart at the ages of 16 and 52 weeks.

PURPOSE: To compare the motor development of patients with hypoplastic left heart syndrome (HLHS) and other types of univentricular heart (UVH) with peers who are healthy at the ages of 16 and 52 weeks. METHODS: Motor development was assessed with the Alberta Infant Motor Scale (AIMS). RESULTS: Both the 23 patients with HLHS and the 13 patients with UVH had lower total AIMS scores in both observations than the controls. At the age of 52 weeks, patients with HLHS had significantly lower scores in all 4 AIMS subscales, whereas patients with UVH had lower scores only in the prone and standing subscales. CONCLUSION: Motor development of patients with HLHS or UVH is delayed during the first year of life, especially in the prone and standing subscales.

Neurodevelopmental burden at age 5 years in patients with univentricular heart.

BACKGROUND: Despite increasing survival, patients with hypoplastic left heart syndrome (HLHS) and other forms of functionally univentricular heart defects (UVHs) remain at increased risk of long-term neurodevelopmental deficits. METHODS: A nationwide sample of 23 patients with HLHS, 13 with UVH, and 40 controls were followed prospectively until the age of 5 years, when neurologic, neuropsychological, and motor examinations and brain MRI were performed. RESULTS: The median full-scale IQ was significantly lower in patients with HLHS (97, P < .001) and patients with UVH (112, P = .024) compared with controls (121). Major neurodevelopmental impairment was found in 26% of the patients with HLHS and 23% of those with UVH, and minor neurologic dysfunction was found in 43% and 46%, respectively. MRI revealed abnormalities, mostly ischemic changes of different degrees, in 82% of the patients with HLHS and in 56% of those with UVH. Prominent changes were significantly associated with neurodevelopmental findings and parental reports of adaptive behavior. In linear regression, significant risk factors for a worse outcome were a history of clinical seizures in connection with the primary operation, a lower diameter of the neonatal ascending aorta, and several pre-, peri-, and postoperative factors related to the primary and bidirectional Glenn operations. CONCLUSIONS: Although median cognitive performance was within the normal range, neurodevelopmental and brain MRI abnormalities were found in the majority of the patients with UVH, and especially in those with HLHS, at preschool age. Both a narrowed ascending aorta and operation-related factors contributed to these findings.

Neurocognitive development and behavioral outcome of 2-year-old children with univentricular heart.

Recent advances in the treatment of children with severe congenital heart defects, such as hypoplastic left heart syndrome (HLHS) and other forms of univentricular heart (UVH), have significantly improved their survival rates. However, these children are at risk for various neurodevelopmental deficits. The aim of the present study was to assess cognitive development, expressive language, and behavior in 30-month-old children with univentricular heart. The participants were 22 children with HLHS, 14 with UVH, and 41 healthy control subjects. The Bayley Scales of Infant Development II, MacArthur Communicative Development Inventories, and Child Behavior Checklist were used for assessments. The results revealed that children with HLHS exhibited a significantly lower mean mental development index, more delays in expressive language functions, and more behavioral problems than did the control children. Two children with HLHS (9%) had mental development indexes below 50, indicating significantly delayed performance. The children with UVH differed from the control children with respect to their lower mean mental development index. These findings suggest that at the age of 30 months, neurodevelopmental deficits are especially prevalent in children with HLHS. Thus, early developmental screening, intervention, and neuropsychological follow-up until school age is recommended particularly for the children with HLHS.

Neurodevelopment in children with hypoplastic left heart syndrome.

OBJECTIVE: To assess neurodevelopment in children with hypoplastic left heart syndrome (HLHS) or other types of functionally single ventricle (univentricular heart, UVH) and to estimate the effect of possible clinical predictors on outcome. STUDY DESIGN: A total of 22 patients with HLHS and 14 with UVH, surviving after palliative surgery performed in the same center, and 42 healthy control subjects were examined at a median age of 30.2 months neurologically and according to the Bayley Scales of Infant Development in a population based prospective neurodevelopmental follow-up study. RESULTS: The mean Mental Developmental Index was significantly lower (89.9) in patients with HLHS than in control subjects (105.5, P<.001), whereas there was no difference between patients with UVH (98.5) and control subjects. The mean Psychomotor Developmental Index in patients with HLHS (80.7, P<.001) as well as in those with UVH (94.5, P=.016) was significantly inferior to that in control subjects (105.3). CONCLUSIONS: In patients with HLHS, mean Mental Developmental Index lags behind control subjects. Psychomotor Developmental Index is impaired in patients with both HLHS and UVH. Routine neurodevelopmental follow-up is recommended for this seriously ill patient group.

Prospective follow-up study of children with univentricular heart: neurodevelopmental outcome at age 12 months.

OBJECTIVES: Despite recent advances in the treatment of children with univentricular heart, their neurodevelopmental outcome remains a major concern. METHODS: This prospective follow-up study evaluated the neurodevelopmental outcome of 23 patients with hypoplastic left heart syndrome, 14 with other forms of univentricular heart, and 46 healthy control subjects at a median age of 12.2 months. The Griffiths Developmental Scale and Alberta Infant Motor Scale served for developmental evaluation. RESULTS: The mean Griffiths developmental quotient of children with hypoplastic left heart syndrome was significantly less (91.6) than that of control children (106.8, P < .001). Patients with univentricular heart scored significantly lower than control subjects only in the gross motor domain (P = .001) but not in overall development (100.6). Alberta Infant Motor Scale scores were significantly lower in children with hypoplastic left heart syndrome (37.5, P < .001) and univentricular heart (43.5, P = .011) than in control subjects (53.3). In linear regression a diagnosis of hypoplastic left heart syndrome (P = .016), a clinical history of seizure (P = .002), and the highest plasma lactate level after the bidirectional Glenn operation (P = .045) were significantly associated with the developmental quotient. CONCLUSIONS: At age 1 year, the level of development of children with univentricular heart was significantly lower than for control subjects only in motor skills, whereas children with hypoplastic left heart syndrome had a more widespread developmental delay. The diagnosis, a clinical seizure history, and increased plasma lactate levels after the bidirectional Glenn operation emerged as risk factors.

Neurodevelopmental and neuroradiologic outcomes in patients with univentricular heart aged 5 to 7 years: related risk factor analysis.

OBJECTIVE: Despite improved survival and neurodevelopmental outcome, children with hypoplastic left heart syndrome and other forms of univentricular heart remain at increased risk for cognitive, motor, and other neurologic deficits. METHODS: We examined 27 children with hypoplastic left heart syndrome or other forms of univentricular heart at a median age of 5.70 years (range 4.99-7.51 years) and performed brain computed tomography or magnetic resonance imaging on 20. Possible risk factors were correlated with outcome. RESULTS: Mean full-scale IQ among patients with hypoplastic left heart syndrome was 86.7; that among patients with other forms of univentricular heart was 89.1, with both differing significantly from the expected population mean (P = .015 and P = .029, respectively). Cerebral palsy was diagnosed in 1 of 7 patients with hypoplastic left heart syndrome and 2 of 20 with other forms of univentricular heart. Brain computed tomography or magnetic resonance imaging revealed ischemic changes and infarcts or atrophy in 5 of 8 patients who had undergone the Norwood procedure and in 2 of 12 of those who had not (P = .062). Abnormal computed tomographic findings correlated significantly with lower full-scale IQ (P = .045) and verbal IQ (P = .02). In the multiple linear regression model, diuresis the third day after the primary operation and cardiopulmonary bypass time in the bidirectional Glenn operation correlated significantly with the primary outcome of full-scale IQ. CONCLUSION: In children with univentricular heart, intellectual and neurologic deficits are common. Perioperative and postoperative risk factors related to the primary phase and bidirectional Glenn operation contribute to these deficits.