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The Neurodevelopmental and Psychological Outcomes Working Group of the Cardiac Neurodevelopmental Outcome Collaborative was formed in 2018 through support from an R13 grant from the National Heart, Lung, and Blood Institute with the goals of identifying knowledge gaps regarding the neurodevelopmental and psychological outcomes of individuals with CHD and investigations needed to advance science, policy, clinical care, and patient/family outcomes. Accurate characterisation of neurodevelopmental and psychological outcomes in children with CHD will drive improvements in patient and family outcomes through targeted intervention. Decades of research have produced a generalised perspective about neurodevelopmental and psychological outcomes in this heterogeneous population. Future investigations need to shift towards improving methods, measurement, and analyses of outcomes to better inform early identification, prevention, and intervention. Improved definition of underlying developmental, neuropsychological, and social-emotional constructs is needed, with an emphasis on symptom networks and dimensions. Identification of clinically meaningful outcomes that are most important to key stakeholders, including patients, families, schools and providers, is essential, specifically how and which neurodevelopmental differences across the developmental trajectory impact stakeholders. A better understanding of the discontinuity and patterns of neurodevelopment across the lifespan is critical as well, with some areas being more impactful at some ages than others. Finally, the field needs to account for the impact of race/ethnicity, socio-economic status, cultural and linguistic diversity on our measurement, interpretation of data, and approach to intervention and how to improve generalisability to the larger worldwide population of patients and families living with CHD.
Infants with single ventricle congenital heart disease demonstrate increasing head growth after bidirectional Glenn; however, the expected growth trajectory has not been well described.
1) We will describe the pattern of head circumference growth in the first year after bidirectional Glenn. 2) We will determine if head growth correlates with motor developmental outcomes approximately 12 months after bidirectional Glenn.
Sixty-nine single ventricle patients underwent bidirectional Glenn between 2010 and 2016. Patients with structural brain abnormalities, grade III–IV intra-ventricular haemorrhage, significant stroke, or obstructive hydrocephalus were excluded. Head circumference and body weight measurements from clinical encounters were evaluated. Motor development was measured with Psychomotor Developmental Index of the Bayley Scales of Infant Development, Third Edition. Generalised estimating equations assessed change in head circumference z-scores from baseline (time of bidirectional Glenn) to 12 months post-surgery.
Mean age at bidirectional Glenn was 4.7 (2.3) months and mean head circumference z-score based on population-normed data was −1.13 (95% CI −1.63, −0.63). Head circumference z-score increased to 0.35 (95% CI −0.20, 0.90) (p < 0.0001) 12 months post-surgery. Accelerated head growth, defined as an increase in z-score of >1 from baseline to 12 months post-surgery, was present in 46/69 (66.7%) patients. There was no difference in motor Psychomotor Developmental Index scores between patients with and without accelerated head growth.
Single ventricle patients demonstrated a significant increase in head circumference after bidirectional Glenn until 10–12 months post-surgery, at which time growth stabilised. Accelerated head growth did not predict sub-sequent motor developmental outcomes.
The Fontan Outcomes Network was created to improve outcomes for children and adults with single ventricle CHD living with Fontan circulation. The network mission is to optimise longevity and quality of life by improving physical health, neurodevelopmental outcomes, resilience, and emotional health for these individuals and their families. This manuscript describes the systematic design of this new learning health network, including the initial steps in development of a national, lifespan registry, and pilot testing of data collection forms at 10 congenital heart centres.
There is a known high prevalence of genetic and clinical syndrome diagnoses in the paediatric cardiac population. These disorders often have multisystem effects, which may have an important impact on neurodevelopmental outcomes. Taken together, these facts suggest that patients and families may benefit from consultation by genetic specialists in a cardiac neurodevelopmental clinic.
This study assessed the burden of genetic disorders and utility of genetics evaluation in a cardiac neurodevelopmental clinic.
A retrospective chart review was conducted of patients evaluated in a cardiac neurodevelopmental clinic from 6 December, 2011 to 16 April, 2013. All patients were seen by a cardiovascular geneticist with genetic counselling support.
A total of 214 patients were included in this study; 64 of these patients had a pre-existing genetic or syndromic diagnosis. Following genetics evaluation, an additional 19 were given a new clinical or laboratory-confirmed genetic diagnosis including environmental such as teratogenic exposures, malformation associations, chromosomal disorders, and single-gene disorders. Genetic testing was recommended for 112 patients; radiological imaging to screen for congenital anomalies for 17 patients; subspecialist medical referrals for 73 patients; and non-genetic clinical laboratory testing for 14 patients. Syndrome-specific guidelines were available and followed for 25 patients with known diagnosis. American Academy of Pediatrics Red Book asplenia guideline recommendations were given for five heterotaxy patients, and family-based cardiac screening was recommended for 23 families affected by left ventricular outflow tract obstruction.
Genetics involvement in a cardiac neurodevelopmental clinic is helpful in identifying new unifying diagnoses and providing syndrome-specific care, which may impact the patient’s overall health status and neurodevelopmental outcome.
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