Psychomotorische und kognitive Entwicklung und Lebensqualität bei Kindern und Jugendlichen mit angeborenem Herzfehler

Hövels-Gürich, Hedwig H.

doi:10.1055/a-0942-1756

Cited by 4 publications

(2 citation statements)

References 84 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Research has also shown that recurrent hypoxia and/ or hypercapnia can play a deleterious role in brain development, mainly in the first years of life, as previously reported in prematurity or congenital heart defects [6,19]. But relationships between neurocognition and severity of respiratory deficits have not been demonstrated in CCHS [30].…”

Section: Discussionmentioning

confidence: 95%

Neurocognition in Congenital Central Hypoventilation Syndrome: influence of genotype and ventilation method

Trang

Bourgeois

Chéliout-Héraut

2020

Orphanet J Rare Dis

View full text Add to dashboard Cite

Background Congenital Central Hypoventilation Syndrome (CCHS) is characterized by central hypoventilation due to abnormal autonomic control of breathing and global dysautonomia. Patients harbour heterozygous PHOX-2B gene mutations which are polyalanine repeats of various lengths in most of the cases. A few previous studies have reported learning difficulties and neuropsychological disorders in patients with CCHS. The aims of the present study were (1) to explore the intellectual abilities of a group of children with CCHS followed up in the centre of reference for CCHS in France using the Wechsler batteries of tests, (2) and to assess whether there was any association between CCHS characteristics and various domains of the intellectual functioning. Results There were 34 consecutive patients (15 males, 19 females) of mean (SD) age of 7.8 (3.8) years, ranging from 4 to 16 years and 6 months. Mean score of full-scale intelligence quotient was 82 (20), being in the low average range. Indexes of working memory and processing speed were significantly lower as compared to the other Wechsler indexes. There were two important findings: (1) full-scale intelligence quotient as well as indexes of verbal comprehension and processing speed were significantly greater in patients with mask ventilation than in those with tracheostomy ventilation (p = 0.012, 0.032 and 0.042 respectively); (2) most interestingly, in the patients with polyalanine repeats mutations, all intellectual indexes negatively correlated with the number of polyalanine expansion, with statistical significance reached for indexes of fluid reasoning and working memory (R = − 0.449, p = 0.032 and R = − 0.562, p = 0.012 respectively). Conclusions CCHS increased the risk to develop neurocognitive deficiencies, affecting particularly speed of processing and working memory. Our results suggested that both genetics and ventilation method could be also involved in the physiopathology of neurocognitive impairment. Further investigations were required to untangle the complex underlying processes. Neurocognitive assessments should be performed regularly in children with CCHS in order to plan re-education programs, adapt school integration and improve quality of life.

show abstract

Section: Discussionmentioning

confidence: 95%

Neurocognition in Congenital Central Hypoventilation Syndrome: influence of genotype and ventilation method

Trang

Bourgeois

Chéliout-Héraut

2020

Orphanet J Rare Dis

View full text Add to dashboard Cite

show abstract

“…The attention level assessment was performed using the first 9 items of the SNAP IV scale [29]. The SNAP IV scale is widely used [30][31][32] for screening attention deficit hyperactivity disorder in children; here, it was applied to parents and/or guardians in an appropriate room.…”

Section: Attention Deficitmentioning

confidence: 99%

Neurodevelopmental Outcomes among Brazilian Children with Cyanotic Congenital Heart Disease and Its Associated Factors

et al. 2022

View full text Add to dashboard Cite

Objectives: The aim of this study was to evaluate neurodevelopmental outcomes (motor development, nonverbal intelligence, and attention) in children with cyanotic congenital heart disease (CHD) compared with healthy children from a public hospital in southern Brazil. Materials and Methods: This was a cross-sectional study with pediatric patients of both sexes: 37 children with cyanotic CHD and a control group with 38 healthy children. Parents/guardians undertook a questionnaire and the SNAP IV scale (to evaluate attention) was applied. Two instruments were applied to each child: the R-2 Non-Verbal Intelligence test and the motor development scale. To assess the factors associated with insufficient performance in the three fields of neurodevelopment, a Poisson regression analysis was performed with a robust estimate. Results: There were no significant differences between children with cyanotic CHD and the control group for any of the neurodevelopmental outcomes studied. Low socioeconomic class was a factor associated with worse performance on the intelligence test and inattention. Furthermore, age was a factor for performance on the intelligence test, while a greater number of siblings was a factor associated with worse performance on the attention test. Conclusions: Public policies regarding child health must involve prioritizing the improvement of families’ social conditions.

show abstract

Socio-demographic parameters and non-cardiac comorbidity related to self-perceived quality of life in young adults after neonatal arterial switch operation for transposition of the great arteries

et al. 2022

View full text Add to dashboard Cite

Evaluating the relation of non-cardiac comorbidity and socio-demographic factors to physical and mental health-related quality of life (QOL) which has been partially found at elevated risk in young adults after neonatal arterial switch operation (ASO) for transposition of the great arteries (TGA). In a prospective reassessment study, results of 92 unselected young adults (22.8 ± 2.6 years) having undergone evaluation of QOL (SF-36) were related to non-cardiac comorbidity with special respect to neurologic and psychiatric comorbidity and to socio-demographic parameters. Neurologic (14%) contrary to psychiatric comorbidities (6.5%) were more frequent than in the general population. The educational level was higher, the rate of unemployment was double as high compared to the average German population. Significant inverse relations (p = 0.006 to 0.033) existed between physical health domains (physical functioning and general health perception) and non-cardiac, neurologic, and psychiatric comorbidity, as well as correlations between the latter domains and socio-economic status, educational level, and worse employment status (Spearman 0.22–0.41, p < 0.0001 to 0.036). Mental health domains (vitality, social functioning, psychical health) were significantly inversely related with neurologic and psychiatric comorbidity (p = 0.002 to 0.048) and correlated with higher educational level (Spearman 0.25, p = 0.019). Neurologic and psychiatric comorbidities and socio-demographic parameters are significant risk factors for a reduced QOL concerning physical and mental health in young adults with TGA after ASO. Standardized QOL measurement should be part of routine screening programs to detect subclinical physical, neurodevelopmental, and psychosocial comorbidity. Graphical abstract

show abstract

Psychomotorische und kognitive Entwicklung und Lebensqualität bei Kindern und Jugendlichen mit angeborenem Herzfehler

Cited by 4 publications

References 84 publications

Neurocognition in Congenital Central Hypoventilation Syndrome: influence of genotype and ventilation method

Neurocognition in Congenital Central Hypoventilation Syndrome: influence of genotype and ventilation method

Neurodevelopmental Outcomes among Brazilian Children with Cyanotic Congenital Heart Disease and Its Associated Factors

Socio-demographic parameters and non-cardiac comorbidity related to self-perceived quality of life in young adults after neonatal arterial switch operation for transposition of the great arteries

Contact Info

Product

Resources

About