Building a sustainable neurocardiac critical care program in a paediatric cardiac ICU: insights and lessons learned.

Introduction: Children with CHD are at increased risk for neurodevelopmental disabilities and neuropsychological impairments throughout their life span. The purpose of this report is to share our experience building a sustainable, novel, inpatient, interdisciplinary Neurocardiac Critical Care Program to mitigate risks and optimize outcomes during the ICU stay.

Material And Methods: A descriptive review was chosen to identify meaningful characteristics, challenges and lessons learned related to the establishment, expansion of and sustainability of Neurocardiac Critical Care Program in a 26-bed pediatric cardiac ICU.

Ketamine Versus Midazolam as the First-Line Continuous Infusion for Status Epilepticus in Children with Cardiac Disease.

Background: The treatment of status epilepticus (SE) in children with cardiac disease is challenging given their often-tenuous hemodynamic state. We aim to determine whether ketamine is safe and effective in children with cardiac disease as the first-line continuous infusion for the treatment of refractory SE (RSE) and to compare ketamine to midazolam for the treatment of RSE in this population.

Methods: This is a single-center retrospective cohort study of pediatric patients with cardiac disease and RSE admitted to the cardiac intensive care unit at a tertiary children's hospital between January 1, 2017 and June 30, 2023.

Multiple Pediatric Extracorporeal Membrane Oxygenation Runs and Futility. What Are the Limits?

Despite high mortality rates, pediatric extracorporeal membrane oxygenation (ECMO) redeployments are frequently discussed in everyday clinical care. We aim to investigate predictors of mortality in those patients. Clinical data from a single pediatric center were retrospectively analyzed.

Hippocampal rotation is associated with ventricular atrial size.

Background: Fetal ventriculomegaly is a source of apprehension for expectant parents and may present prognostic uncertainty for physicians. Accurate prenatal counseling requires knowledge of its cause and associated findings as the differential diagnosis is broad. We have observed an association between ventriculomegaly and incomplete hippocampal inversion.

[Formula: see text] Trajectories of neurodevelopment and opportunities for intervention across the lifespan in congenital heart disease.

Children with congenital heart disease (CHD) are at increased risk for neurodevelopmental challenges across the lifespan. These are associated with neurological changes and potential acquired brain injury, which occur across a developmental trajectory and which are influenced by an array of medical, sociodemographic, environmental, and personal factors. These alterations to brain development lead to an array of adverse neurodevelopmental outcomes, which impact a characteristic set of skills over the course of development.

Pregnancy and Child Outcomes Following Fetal Intracranial Hemorrhage.

Background: The prenatal and early postnatal outcomes of fetal intracranial hemorrhage (ICH) prenatally diagnosed by fetal magnetic resonance imaging (MRI) have not been well described.

Methods: A retrospective study of cases with fetal ICH diagnosed by fetal MRI at Children's National Hospital, Washington, DC, from 2012 to 2020 was conducted. Maternal characteristics, prenatal imaging, pregnancy outcome, and child developmental outcomes were recorded.

Autonomic markers of extubation readiness in premature infants.

Background: In premature infants, extubation failure is common and difficult to predict. Heart rate variability (HRV) is a marker of autonomic tone. Our aim is to test the hypothesis that autonomic impairment is associated with extubation readiness.

Isolated Absent Septum Pellucidum: A Retrospective Study of Fetal Diagnosis and Postnatal Outcomes.

Background: Absent septum pellucidum (ASP) is a brain abnormality often associated with neuroanatomic abnormalities including septo-optic dysplasia (SOD). We aimed to determine how frequently prenatally diagnosed isolated ASP is confirmed by postnatal imaging and to examine clinical outcomes for ASP.

Methods: This was a retrospective study of maternal-fetal dyads referred to Children's National Hospital from January 1, 2012, to June 30, 2019.

Outcome of Agenesis of the Corpus Callosum Diagnosed by Fetal MRI.

Background: Fetal magnetic resonance imaging (MRI) is increasingly utilized for prenatal diagnosis of agenesis of the corpus callosum (ACC). This study aimed to (1) describe cases of ACC diagnosed by fetal MRI, (2) determine the frequency of postnatal confirmation by MRI, and (3) understand postnatal outcomes of infants with ACC.

Methods: Maternal records from Children's National Hospital between January 2012 and June 2019 with a prenatal neurological consultation, fetal MRI, and ACC on imaging were included.

Fetal brain MRI: neurometrics, typical diagnoses, and resolving common dilemmas.

This review presents a practical approach to imaging the fetal brain by MRI. Herein, we demonstrate how to measure brain structures and fluid spaces, and discuss the importance of comparing measurements to normative biometric references at a corresponding gestational age. We present some common imaging dilemmas of the technical aspects of fetal MRI with regard to typical regions of abnormality including the cerebrum, the ventricular system, and the posterior fossa, and discuss how to resolve them.

In infants with congenital heart disease autonomic dysfunction is associated with pre-operative brain injury.

Background: Brain injury is a serious and common complication of critical congenital heart disease (CHD). Impaired autonomic development (assessed by heart rate variability (HRV)) is associated with brain injury in other high-risk neonatal populations.

Objective: To determine whether impaired early neonatal HRV is associated with pre-operative brain injury in CHD.

Taenia-tela choroidea complex and choroid plexus location help distinguish Dandy-Walker malformation and Blake pouch cysts.

Background: Dandy-Walker malformation and Blake pouch cysts can have overlapping imaging features. The choroid plexus and associated taenia-tela choroidea complex are displaced inferolaterally in Dandy-Walker malformation and below the vermis in Blake pouch cysts.

Objective: To determine the normal fetal and postnatal MR appearance of the choroid plexus and taenia-tela choroidea complex, and whether their location can help distinguish Dandy-Walker malformation from Blake pouch cysts.

Autonomic development in preterm infants is associated with morbidity of prematurity.

Background: Previous studies have described an association between preterm birth and maturation of the autonomic nervous system (ANS); however, this may be impacted by multiple factors, including prematurity-related complications. Our aim was to evaluate for the effect of prematurity-related morbidity on ANS development in preterm infants in the NICU.

Methods: We compared time and frequency domains of heart rate variability (HRV) as a measure of ANS tone in 56 preterm infants from 2 NICUs (28 from each).

The Role of Fetal MRI for Suspected Anomalies of the Posterior Fossa.

Background: Posterior fossa anomalies can be diagnostic dilemmas during the fetal period. The prognosis for different diagnoses of the posterior fossa varies widely. We investigated whether fetal magnetic resonance imaging (MRI) and prenatal neurology consultation led to an alternate prognosis for fetuses referred due to concern for a fetal posterior fossa anomaly and concordance between pre- and postnatal diagnoses.

Exposures influencing the developing central autonomic nervous system.

Autonomic nervous system function is critical for transition from in-utero to ex-utero life and is associated with neurodevelopmental and neuropsychiatric outcomes later in life. Adverse prenatal and neonatal conditions and exposures can impair or alter ANS development and, as a result, may also impact long-term neurodevelopmental outcomes. The objective of this article is to provide a broad overview of the impact of factors that are known to influence autonomic development during the fetal and early neonatal period, including maternal mood and stress during and after pregnancy, fetal growth restriction, congenital heart disease, toxic exposures, and preterm birth.

Autonomic nervous system maturation in the premature extrauterine milieu.

Background: In premature infants, we investigated whether the duration of extrauterine development influenced autonomic nervous system (ANS) maturation.

Methods: We performed a longitudinal cohort study of ANS maturation in preterm infants. Eligibility included birth gestational age (GA) < 37 weeks, NICU admission, and expected survival.

How prenatal head ultrasound reference ranges affect evaluation of possible fetal microcephaly.

Objectives: Different fetal ultrasound (US) nomograms of the head circumference (HC) have been established; however, comparisons between the detection rates of microcephaly among US nomograms are few and inconsistent. We aimed to compare the prenatal diagnostic rate of fetal microcephaly (FM) among four widely used US nomograms of the fetal HC, when applied to the same group of fetuses.

Methods: We retrospectively identified singleton pregnancies complicated by fetal HC < 5th percentile for gestational age (GA) by US, without other risk factors for FM and with normal fetal brain MRI.

Placental Pathology and Neuroimaging Correlates in Neonates with Congenital Heart Disease.

Congenital heart disease (CHD) is an independent risk factor for brain injury, including stroke, and poor neurodevelopmental outcomes, and placental abnormalities may represent an additional risk factor for brain injury in neonates. The incidence and scope of placental pathology and relationship to fetal brain abnormalities in pregnancies complicated by fetal CHD has not been explored to our knowledge. In order to determine the prevalence of placental pathology findings and whether placental findings are associated with postnatal brain injury in pregnancies complicated by fetal CHD, we reviewed placental pathology reports for 51 pregnancies complicated by CHD and scored available postnatal, pre-operative brain MRI for brain pathology.

Neuronal c-Abl activation leads to induction of cell cycle and interferon signaling pathways.

Background: Expression of active c-Abl in adult mouse forebrain neurons in the AblPP/tTA mice resulted in severe neurodegeneration, particularly in the CA1 region of the hippocampus. Neuronal loss was preceded and accompanied by substantial microgliosis and astrocytosis. In contrast, expression of constitutively active Arg (Abl-related gene) in mouse forebrain neurons (ArgPP/tTA mice) caused no detectable neuronal loss or gliosis, although protein expression and kinase activity were at similar levels to those in the AblPP/tTA mice.

c-Abl in neurodegenerative disease.

The c-Abl tyrosine kinase participates in a variety of cellular functions, including regulation of the actin cytoskeleton, regulation of the cell cycle, and the apoptotic/cell cycle arrest response to stress, and the Abl family of kinases has been shown to play a crucial role in development of the central nervous system. Recent studies have shown c-Abl activation in human Alzheimer's and Parkinson's diseases and c-Abl activation in mouse models and neuronal culture in response to amyloid beta fibrils and oxidative stress. Overexpression of active c-Abl in adult mouse neurons results in neurodegeneration and neuroinflammation.

Neuronal c-Abl overexpression leads to neuronal loss and neuroinflammation in the mouse forebrain.

Several immunocytochemical studies have revealed that Abelson tyrosine kinase (c-Abl) is associated with both neuritic plaques and neurofibrillary tangles in the brains of patients with Alzheimer's disease (AD). Additionally, c-Abl has been shown to phosphorylate tau on tyrosine 394. The activity of c-Abl is also involved in the control of the cell cycle and apoptosis.