Neurodevelopmental outcome of patients after the Fontan operation: A comparison between children with hypoplastic left heart syndrome and other functional single ventricle lesions

Goldberg, Caren S.; Schwartz, Edward; Brunberg, James A.; Mosca, Ralph S.; Bove, Edward L.; Schork, M. Anthony; Stetz, Sharon P.; Cheatham, John P.; Kulik, Thomas J.

doi:10.1067/mpd.2000.108952

Cited by 209 publications

(147 citation statements)

References 20 publications

Supporting

Mentioning

138

Contrasting

Unclassified

Order By: Relevance

“…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).…”

mentioning

confidence: 90%

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

et al. 2017

View full text Add to dashboard Cite

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

show abstract

mentioning

confidence: 90%

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

et al. 2017

View full text Add to dashboard Cite

show abstract

“…4,[8][9][10][11][12] Our report evaluated the risk for hospital mortality in infants born with major CHDs who were supported with intensive care. In all, 25 (26%) of the infants died before undergoing surgical procedures, similar to that reported in Fesslova et al 3 …”

Section: Discussionmentioning

confidence: 99%

The effect of late preterm birth on mortality of infants with major congenital heart defects

et al. 2011

View full text Add to dashboard Cite

Objective: We evaluated the effect of late preterm delivery (34 to 36 weeks) on hospital mortality of infants with congenital heart defects (CHDs).Study Design: Retrospective record review of infants with major CHD born at or after to 34 weeks, cared for in a single tertiary perinatal center between 2002 and 2009. Factors associated with death before discharge from the hospital were ascertained using univariate and multivariate analyses.Result: Of the 753 infants with CHD, 117 were born at late preterm. Using logistic regression analysis, white race (OR; 95% CI) (0.60; 0.39 to 0.95), late preterm delivery (2.70; 1.69 to 4.33), and need for intubation in the delivery room (3.15; 1.92 to 5.17) were independently associated with hospital death. Conclusion:Late preterm birth of infants with major CHDs was independently associated with increased risk of hospital death compared with delivery at more mature gestational ages.

show abstract

“…[4][5]7,8,10 In addition, the patients with HLHS who had deep hypothermic circulatory arrest during their first-stage surgical palliation were noted to have worse outcomes. 5,7,8 Mahle et al 4 evaluated outcomes in school-aged adolescents with HLHS and identified IQ scores within normal range with mean performance of the group lower than that of the general population. Of note, the majority of parents or guardians in this study described their child's health as good (34%) or excellent (45%) and their academic performance as average (42%) or above average (42%).…”

Section: Neurodevelopmental and Cognitive Outcomesmentioning

confidence: 99%

“…The majority of authors identified similar neurodevelopmental outcomes, but variations were noted depending on the severity of the SV anatomy and sample selection. 4,5,7,8 Uzark et al 8 provided one of the first neurodevelopmental outcome studies in the population who have undergone the Fontan operation. Neurodevelopmental testing in patients 2 to 16 years of age found that the majority of children had intellectual function within the normal range.…”

Section: Neurodevelopmental and Cognitive Outcomesmentioning

confidence: 99%

“…Past research in patients with SV CHD has focused on functional and neurodevelopmental outcomes producing a growing body of literature with documented deficits that seem to be multifactoral. [4][5][6][7][8][9][10] However, little is known about the other determinants of HRQOL such as social well-being and function. Moreover, earlier studies in this population had identified the perceptions of family members rather than the actual self-perceived HRQOL of the individual, who may not have been of sufficient age to participate in the evaluation.…”

mentioning

confidence: 99%

See 1 more Smart Citation

Health-related quality of life: A closer look at related research in patients who have undergone the Fontan operation over the last decade

et al. 2007

View full text Add to dashboard Cite

Health-related quality of life: A closer look at related research in patients who have undergone the Fontan operation over the last decade The advancements in surgical technique and perioperative care have significantly improved the survival of children with single ventricle (SV) congenital heart disease (CHD) over the past decade. The population who have undergone the Fontan operation are growing into adulthood and facing many unique challenges. Past research has focused on functional and neurodevelopmental outcomes with inferences made to health-related quality of life (HRQOL). With the population who have undergone the Fontan operation surviving into adulthood, little research has been directed toward the self-report of HRQOL in adolescents and young adults after surgical palliation. Questions still remain on how these patients will transition into adulthood and whether they will live normal productive lives. This article reviews the literature related to HRQOL in the SV subgroup of CHD. In addition, an overview of newly developed disease-specific HRQOL instruments is presented as well as limitations and future research in HRQOL of the SV Fontan population.

show abstract

Neurodevelopmental outcome of patients after the Fontan operation: A comparison between children with hypoplastic left heart syndrome and other functional single ventricle lesions

Cited by 209 publications

References 20 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

The effect of late preterm birth on mortality of infants with major congenital heart defects

Health-related quality of life: A closer look at related research in patients who have undergone the Fontan operation over the last decade

Contact Info

Product

Resources

About