Neurodevelopmental Burden at Age 5 Years in Patients With Univentricular Heart

Sarajuuri, Anne; Jokinen, Eero; Mildh, Leena; Tujulin, Anna-Mari; Mattila, Ilkka; Valanne, Leena; Lönnqvist, Tuula

doi:10.1542/peds.2012-0486

Cited by 53 publications

(51 citation statements)

References 26 publications

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“…Patients with SV require multiple surgeries, often beginning with the stage 1 Norwood procedure in the first few weeks of life, and leading to the Fontan procedure generally between 1.5 and 4 y of age (3). Post-Fontan brain MRI studies of SV patients are few, but suggest that evidence of brain injury is still present in some patients into childhood (14,15). Recently, we reported increased number of gross anatomic brain abnormalities by MRI in our current sample of SV patients 10-19 y of age as compared to a referent sample (7).…”

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confidence: 61%

Reduced cortical volume and thickness and their relationship to medical and operative features in post-Fontan children and adolescents

et al. 2017

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Background:We compared brain cortical and subcortical gray matter volumes and cortical thickness between postFontan patients and healthy controls, and examined brain anatomical associations with operative and medical history characteristics. Methods: Post-Fontan (n = 128 volumes; n = 115 thickness) and control subjects (n = 48 volumes; n = 45 thickness) underwent brain MRI at ages 10-19 y. Subcortical and cortical volumes and cortical thicknesses were measured for intergroup comparison. Associations between brain measures and clinical measures were assessed in the Fontan group. results: Widespread, significant reduction in brain volumes and thicknesses existed in the Fontan group compared to controls, spanning all brain lobes and subcortical gray matter. Fontan subjects treated with vs. without the Norwood procedure had smaller volumes in several terminal clusters, but did not differ in cortical thickness. Older age at first operation and increasing numbers of cardiac catheterizations, operative complications, and catheterization complications were associated with lower regional volumes and thicknesses. Increasing numbers of operative complications and cardiac catheterizations were associated with smaller regional volumes in the Norwood group. conclusion: The post-Fontan adolescent brain differs from the normal control brain. Some of these differences are associated with potentially modifiable clinical variables, suggesting that interventions might improve long-term neurocognitive outcome.c ongenital heart disease (CHD) is the most common congenital anomaly, with single ventricle (SV) defects occurring in nearly 10% of patients with CHD (1). Patients with SV, particularly hypoplastic left heart syndrome (HLHS), have higher mortality and morbidity than those with other types of CHD, but their survival has steadily increased for several decades (2). The Fontan procedure, as well as staged palliation

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confidence: 61%

Reduced cortical volume and thickness and their relationship to medical and operative features in post-Fontan children and adolescents

et al. 2017

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“…In a cohort of subjects with hypoplastic left heart syndrome assessed at 12 months, 30 months, and 5 years of age, early outcomes were significantly associated with scores achieved at 5 years. 17 In subjects with repaired transposition of the great arteries, developmental achievement at 1 year of age was only modestly associated with outcomes at 8 years. 18 This study allowed direct comparison of similar-aged children with 1V anatomy to those with 2V anatomy.…”

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confidence: 99%

Risk and Prevalence of Developmental Delay in Young Children With Congenital Heart Disease

et al. 2014

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WHAT'S KNOWN ON THIS SUBJECT: Children with congenital heart disease demonstrate a high prevalence of low-severity developmental problems in the areas of language, motor skills, attention, and executive function. Systematic evaluation has been recommended to promote early detection of problems and ensure appropriate intervention. WHAT THIS STUDY ADDS:This study presents results of longitudinal testing in early childhood. Developmental delays were common. Feeding difficulty and medical and genetic comorbidities increased risk for delays. Exposure to risk and prevalence of delay change over time; therefore, repeated evaluations are warranted.abstract BACKGROUND AND OBJECTIVE: Children with congenital heart disease (CHD) are at risk for developmental delay (DD). Changes in cognitive, language, and motor skills in early childhood have not been described. We report the results of a structured approach using longitudinal testing to identify problems and ensure early intervention in accordance with published guidelines.METHODS: Bayley Scales of Infant Development, Third Edition, were used to assess cognitive, language, and motor skills in 99 children with CHD. Subjects were evaluated 3 to 6 times in the first 3 years of life. DD was defined as scores .1 SD below the population mean. RESULTS:Cardiac anatomy was single ventricle (1V) in 34 subjects and 2 ventricles (2V) in 65. Medical comorbidities were present in 21% and genetic syndromes in 19%. Most subjects (75%) had DD in $1 area at $1 assessments. Subjects with 1V anatomy had equivalent outcomes to those with 2V. Cognitive and language scores declined in subjects with genetic syndromes but were stable and within the average range for subjects with 1V and 2V. Motor scores improved for subjects with 1V and 2V but remained low for those with genetic syndromes. In addition to age, need for supplemental tube feeding, longer cardiopulmonary bypass time, and shorter time since last hospitalization were significant predictors of developmental outcomes.CONCLUSIONS: DDs in young children with CHD are both common and dynamic. Providers should encourage longitudinal surveillance for children with CHD because exposure to risk and prevalence of DD change over time. Pediatrics 2014;133:e570-e577 AUTHORS:

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“…Major neuro-developmental impairment was found in 26% and minor neurologic dysfunction in 43% of the patients with HLHS. Magnetic resonance imaging revealed cerebral abnormalities, mostly ischemic changes of different degrees, in 82% of the patients [21]. Major neurologic dysfunction was found in 19% of our patients ranging from autism to epilepsy.…”

Section: Discussionmentioning

confidence: 67%

Long-term results of children operated for hypoplastic left heart syndrome in Children's Heart Centre

Vojtovič¹,

Tláskal²,

Gebauer³

et al. 2014

Cor Vasa

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c o r e t v a s a 5 6 ( 2 0 1 4 ) e 4 4 9 -e 4 5 5 Children Long-term follow up a b s t r a c t Introduction: Hypoplastic left heart syndrome (HLHS), one of the most serious congenital heart defects, can be surgically paliated using 3 subsequent stages. Long-term results of this approach are reviewed in this study. Methods: Fifty-two consecutive patients (pts.) operated for HLHS from 1999 to 2012 were evaluated retrospectively. Results: Norwood stage I operation was performed at the median age of 6.5 days with a total mortality of 19%. Significant risk factor for death was lower weight at surgery (Cox proportional risk per 1 g increase = 0.997, CI 0.995-0.990, P < 0.001). Between stages I and II, 15 catheter/surgical reintervention had to be carried out in 13 pts. (aortic arch narrowing in 10/13). Forty-two pts. underwent stage II operation at the median age of 6.8 months with a total mortality of 4.8% and 18 subsequent reinterventions in 13 patients. Finally, 26 patients aged median 3.9 years underwent stage III operation (total cavopulmonary connection) with a total mortality of 8%. The probability of survival at 1/5/10 years of age was 77/77/71%. At long-term follow up (median 7.8 years) 37 of the 38 surviving patients are in NYHA functional class I or II. Conclusions: Despite a highly centralized care, surgical treatment of HLHS is still associated with a significant mortality and morbidity. Long-term survivals, however, have an acceptable functional status during childhood corresponding to other groups of patients after surgical palliation for functionally single ventricle.

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Neurodevelopmental Burden at Age 5 Years in Patients With Univentricular Heart

Cited by 53 publications

References 26 publications

Reduced cortical volume and thickness and their relationship to medical and operative features in post-Fontan children and adolescents

Reduced cortical volume and thickness and their relationship to medical and operative features in post-Fontan children and adolescents

Risk and Prevalence of Developmental Delay in Young Children With Congenital Heart Disease

Long-term results of children operated for hypoplastic left heart syndrome in Children's Heart Centre

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