Paediatric rotations in undergraduate medical education in Switzerland: Meeting students' expectations and the goals of the competency-based learning catalogue PROFILES.

Introduction: The competency-based catalogue of learning objectives "Principal Relevant Objectives and Framework for Integrative Learning and Education in Switzerland" (PROFILES) based on Entrustable Professional Activities (EPAs) was newly introduced in 2018 in undergraduate medical education in Switzerland. Clerkships provide opportunities for students to train clinical skills and competencies within the curriculum. This study aims to assess the students' experiences during paediatric clerkships and whether they achieve the expected competency level of certain EPAs by the end of their training.

Learning from those who thrive: protective factors and neuroimaging markers in adolescents with complex congenital heart disease and with a favorable neurodevelopmental profile.

Patients with complex congenital heart disease (cCHD) are at risk for neurodevelopmental impairments, yet many patients develop normally. This study investigated associations between a favorable neurodevelopmental profile and protective factors, quality of life (QoL), resilience, and brain development. Adolescents with cCHD ( = 100) were prospectively enrolled.

Elevated cerebral perfusion in neonatal encephalopathy is associated with neurodevelopmental impairments.

Background: Neonatal encephalopathy (NE) represents a primary cause of neonatal death and neurodevelopmental impairments. In newborns with NE, cerebral hyperperfusion is related to an increased risk of severe adverse outcomes, but less is known about the link between perfusion and mild to moderate developmental impairments or developmental delay.

Methods: Using arterial spin labelling perfusion MRI, we investigated the link between perfusion in 36 newborns with NE and developmental outcome at 2 years.

Early High-Dose Erythropoietin and Cognitive Functions of School-Aged Children Born Very Preterm.

Importance: Children born very preterm are at risk for long-term neurodevelopmental sequelae. Prophylactic high-dose recombinant human erythropoietin (rhEpo) shortly after birth has not been shown to improve cognitive, motor, and behavioral development at 2 and 5 years.

Objective: To investigate whether early high-dose rhEpo is associated with better executive functions and processing speed-late-maturing cognitive functions-in school-aged children born very preterm.

Impact of postoperative necrotizing enterocolitis after neonatal cardiac surgery on neurodevelopmental outcome at 1 year of age.

Objectives: We analyzed the impact of postoperative necrotizing enterocolitis (NEC) after cardiac surgery in neonatal age on neurodevelopmental (ND) outcome at 1 year of age.

Methods: Using data from the Swiss Neurodevelopmental Outcome Registry for Children with Congenital Heart Disease (ORCHID), we analyzed perioperative variables including postoperative NEC (Bell's stage ≥2) and 1-year ND outcome (Bayley III).

Results: The included patients ( = 101) had congenital heart disease (CHD), categorized as follows: 77 underwent biventricular repair for CHD with two functional chambers, 22 underwent staged palliation until the Fontan procedure for CHD with single ventricle physiology ( = 22), or 4 underwent single ventricle palliation or biventricular repair for borderline CHD ( = 4).

Heart rate variability and cognitive functions in adolescents with complex congenital heart disease.

Background: Heart Rate Variability (HRV) originates from the interplay between parasympathetic/sympathetic inputs to the heart, thus serving as an indicator of Autonomic Nervous System regulation. Prior research indicates that decreased HRV, marked by reduced autonomic balance, is related to poorer cognitive performance. While the population with congenital heart disease (CHD) show changes in HRV linked with the heart defect, the association between HRV and cognitive functions in CHD remains unexplored.

Inhibition abilities and functional brain connectivity in school-aged term-born and preterm-born children.

Background: Inhibition abilities are known to have impact on self-regulation, behavior, and academic success, and they are frequently impaired in children born preterm. We investigated the possible contributions of resting-state functional brain connectivity to inhibition following preterm birth.

Methods: Forty-four preterm and 59 term-born participants aged 8-13 years were administered two inhibition tasks and resting-state functional MRI was performed.

Processing Speed Partially Mediates Executive Function Impairments in Adolescents with Congenital Heart Disease: Results from a Prospective Cohort Study.

Objective: To assess processing speed, fine motor function, attention, and executive function (EF) impairments in adolescents with complex congenital heart disease (CHD) who underwent open-heart surgery during infancy.

Study Design: We administered a comprehensive neuropsychological test battery evaluating 5 EF domains: working memory, inhibition, cognitive flexibility, fluency, and planning and primary neurodevelopmental processes (PNPs): processing speed, fine motor function, and attention. The sample included 100 adolescents with complex CHD from a previous University Children's Hospital Zurich study, with 104 healthy controls for comparison.

Effects of hemodynamic alterations and oxygen saturation on cerebral perfusion in congenital heart disease.

Background: Patients with severe congenital heart disease (CHD) are at risk for neurodevelopmental impairment. An abnormal cerebral blood supply caused by the altered cardiac physiology may limit optimal brain development. The aim of this study was to evaluate the effect of a systemic-to-pulmonary shunt, aortic arch obstruction and arterial oxygen saturation on cerebral perfusion in patients with severe CHD.

Motor function outcomes in children with open prenatal repair of Spina Bifida Aperta at 36-month follow-up: The Zurich cohort.

Purpose: This study aimed to describe outcomes of motor function with a special focus on ambulation ability at 36 months among children with open prenatal repair of spina bifida aperta (SB).

Methods: A prospective cohort study was conducted including 87 patients with open prenatal repair of SB at the investigating center born between 2010 and 2018. Anatomic lesion level and motor function level in the neonatal period, as well as motor function level, ambulation status, and use of orthotics and assistive devices at 36 months were assessed.

Cerebral perfusion differences are linked to executive function performance in very preterm-born children and adolescents.

Children and adolescents born very preterm are at risk of cognitive impairment, particularly affecting executive functions. To date, the neural correlates of these cognitive differences are not yet fully understood, although converging evidence points to a pattern of structural and functional brain alterations, including reduced brain volumes, altered connectivity, and altered brain activation patterns. In very preterm neonates, alterations in brain perfusion have also been reported, but the extent to which these perfusion alterations persist into later childhood is not yet known.

Perioperative Brain Injury in Relation to Early Neurodevelopment Among Children with Severe Congenital Heart Disease: Results from a European Collaboration.

Objective: To examine the relationship between perioperative brain injury and neurodevelopment during early childhood in patients with severe congenital heart disease (CHD).

Study Design: One hundred and seventy children with CHD and born at term who required cardiopulmonary bypass surgery in the first 6 weeks after birth were recruited from 3 European centers and underwent preoperative and postoperative brain MRIs. Uniform description of imaging findings was performed and an overall brain injury score was created, based on the sum of the worst preoperative or postoperative brain injury subscores.

Thalamic connectivity topography in newborns with spina bifida: association with neurological functional level but not developmental outcome at 2 years.

Spina bifida affects spinal cord and cerebral development, leading to motor and cognitive delay. We investigated whether there are associations between thalamocortical connectivity topography, neurological function, and developmental outcomes in open spina bifida. Diffusion tensor MRI was used to assess thalamocortical connectivity in 44 newborns with open spina bifida who underwent prenatal surgical repair.

Multimodal personalised executive function intervention (E-Fit) for school-aged children with complex congenital heart disease: protocol for a randomised controlled feasibility study.

Introduction: Children with congenital heart disease (CHD) are at risk for executive functions (EF) impairments. To date, interventions have limited effects on EF in children and adolescents with complex CHD. Therefore, we developed a new multimodal and personalised EF intervention (E-Fit).

Long-term neurodevelopmental outcome and serial cerebral magnetic resonance imaging assessment in Fontan patients at school age.

Objectives: Children with univentricular congenital heart disease undergoing staged surgical palliation are at risk for impaired neurodevelopmental (ND) outcome. Little is known about the long-term effects on brain growth until school age.

Methods: In a prospective two-centre study, consecutive patients undergoing stage I (Hybrid or Norwood) to stage III (Fontan procedure) were evaluated by 2 serial cerebral magnetic resonance imaging examinations, somatic growth and ND testing before Fontan procedure at 2 years of age (Bayley-III) and after Fontan at 6-8 years of age (Wechsler Intelligence Scale for Children-third edition).

Association of cerebellar volume with cognitive and motor function in adults with congenital heart disease.

Introduction: Patients with congenital heart disease (CHD) are at risk for cognitive and motor function impairments, brain injury, and smaller total brain volumes. The specific vulnerability of the cerebellum and its role in cognitive and motor functions in adults with congenital heart disease is not well defined.

Methods: Forty-three patients with CHD and 53 controls between 18 and 32 years underwent brain magnetic resonance imaging and cognitive, executive (EF), and motor function assessment.

Lesion Extent Negatively Impacts Intellectual Skills in Pediatric Focal Epilepsy.

Background: Cognitive development in children and adolescents with focal lesional epilepsy is determined by the underlying epileptogenic lesion, in addition to epilepsy itself. However, the impact of lesion-related variables on intelligence quotient (IQ) and developmental quotient (DQ) remains largely unexplored. Here, we aimed to determine the effect of lesion-related predictors and their relation with epilepsy-related predictors of intellectual functioning.

Mental health-related quality of life in mothers of children with surgically repaired congenital heart disease: a 13-year longitudinal study.

Aims: Having a child with congenital heart disease (CHD) can affect parental health-related quality of life (HR-QoL). We investigated the long-term trajectories of mental HRQoL (m-HRQoL) in mothers of children with CHD and examined risk factors for persistent low m-HRQoL.

Methods: One hundred twenty-five mothers of children with CHD completed a standardized questionnaire on m-HRQoL (mental subscale SF-12) after the children's first open-heart surgery and subsequently when the children were 1, 4, 6, 10, and 13 years old.

Atypical gaze-following behaviour in infants with congenital heart disease.

Background: Neurodevelopmental impairments are the most prevalent non-cardiac long-term sequelae in children with complex congenital heart disease (CHD). Deficits include the social-emotional and social-cognitive domains. Little is known about the predecessors of social-cognitive development in infants with CHD during the first year of life.

Counting on random number generation: Uncovering mild executive dysfunction in congenital heart disease.

Congenital heart disease (CHD) is associated with various neurocognitive deficits, particularly targeting executive functions (EFs), of which random number generation (RNG) is one indicator. RNG has, however, never been investigated in CHD. We administered the Mental Dice Task (MDT) to 67 young adults with CHD and 55 healthy controls.