Regional paediatric cardiac neurodevelopmental care: a survey of New England referral practices and institutional resources.

Background: Reports of paediatric cardiac neurodevelopmental programmes are predominately limited to high-volume surgical centres. Regional characterisation of neurodevelopmental care practices across multiple centres both with and without cardiac surgery programmes has not yet been described.

Objective: Assess cardiac neurodevelopmental infrastructure and approach across New England to describe regional support for children with CHD.

Neurodevelopmental Outcomes in Early Adolescence: The Pediatric Heart Network Single Ventricle Reconstruction Trial.

Background: Neurodevelopmental and functional impairments are among the most consequential morbidities for survivors of hypoplastic left heart syndrome after staged single ventricle surgical palliation. The SVRIII trial (Long-Term Outcomes of Children With Hypoplastic Left Heart Syndrome and the Impact of Norwood Shunt Type) enrolled adolescents, who were randomized to different surgical shunt types at the time of Norwood procedure as neonates, for multifaceted in-person evaluation. This study reports their neurodevelopmental outcomes.

Genome sequencing is critical for forecasting outcomes following congenital cardiac surgery.

While exome and whole genome sequencing have transformed medicine by elucidating the genetic underpinnings of both rare and common complex disorders, its utility to predict clinical outcomes remains understudied. Here, we use artificial intelligence (AI) technologies to explore the predictive value of whole exome sequencing in forecasting clinical outcomes following surgery for congenital heart defects (CHD). We report results for a prospective observational cohort study of 2,253 CHD patients from the Pediatric Cardiac Genomics Consortium with a broad range of complex heart defects, pre- and post-operative clinical variables and exome sequencing.

Non-rotation of the Intestine: A Rare and Unexpected Cause of Bowel Obstruction in an Octogenarian.

An 85-year-old male presented to our emergency department with a three-day history of nausea, vomiting, diffuse lower abdominal pain, and diarrhea. His last bowel movement was in the morning prior to the evaluation. Physical exam demonstrated diffuse abdominal tenderness without peritoneal signs.

Noncoding variants and sulcal patterns in congenital heart disease: Machine learning to predict functional impact.

Neurodevelopmental impairments associated with congenital heart disease (CHD) may arise from perturbations in brain developmental pathways, including the formation of sulcal patterns. While genetic factors contribute to sulcal features, the association of noncoding variants (ncDNVs) with sulcal patterns in people with CHD remains poorly understood. Leveraging deep learning models, we examined the predicted impact of ncDNVs on gene regulatory signals.

Current state of neurodevelopmental and psychosocial care practices for paediatric patients with ventricular assist devices: an Advanced Cardiac Therapies Improving Outcomes Network and Cardiac Neurodevelopmental Outcome Collaborative collaborative survey.

Purpose: Paediatric patients with heart failure requiring ventricular assist devices are at heightened risk of neurologic injury and psychosocial adjustment challenges, resulting in a need for neurodevelopmental and psychosocial support following device placement. Through a descriptive survey developed in collaboration by the Advanced Cardiac Therapies Improving Outcomes Network and the Cardiac Neurodevelopmental Outcome Collaborative, the present study aimed to characterise current neurodevelopmental and psychosocial care practices for paediatric patients with ventricular assist devices.

Method: Members of both learning networks developed a 25-item electronic survey assessing neurodevelopmental and psychosocial care practices specific to paediatric ventricular assist device patients.

Preclinical translational platform of neuroinflammatory disease biology relevant to neurodegenerative disease.

Neuroinflammation is a key driver of neurodegenerative disease, however the tools available to model this disease biology at the systems level are lacking. We describe a translational drug discovery platform based on organotypic culture of murine cortical brain slices that recapitulate disease-relevant neuroinflammatory biology. After an acute injury response, the brain slices assume a chronic neuroinflammatory state marked by transcriptomic profiles indicative of activation of microglia and astrocytes and loss of neuronal function.

"Winged" Eagle's syndrome: neurophysiological findings in a rare cause of spinal accessory nerve palsy. Illustrative cases.

Background: Eagle's syndrome (ES) classically describes dysphagia, globus sensation, and otalgia from an elongated and calcified styloid process or stylohyoid ligament. Compression of the spinal accessory nerve (SAN) has not been reported as an associated feature of ES or related variants.

Observations: The authors describe two cases of an atypical "winged" variant with SAN palsy resulting from compression by a posteriorly angulated or calcified styloid process.

Assessment, patient selection, and rehabilitation of nerve transfers.

Peripheral nerve injuries are common and can have a devastating effect on physical, psychological, and socioeconomic wellbeing. Peripheral nerve transfers have become the standard of care for many types of peripheral nerve injury due to their superior outcomes relative to conventional techniques. As the indications for, and use of, nerve transfers expand, the importance of pre-operative assessment and post-operative optimization increases.

Association of genetic and sulcal traits with executive function in congenital heart disease.

Objective: Persons with congenital heart disease (CHD) are at increased risk of neurodevelopmental disabilities, including impairments to executive function. Sulcal pattern features correlate with executive function in adolescents with single-ventricle heart disease and tetralogy of Fallot. However, the interaction of sulcal pattern features with genetic and participant factors in predicting executive dysfunction is unknown.

The hand diagram: A novel outcome measure following supercharged end-to-side anterior interosseous nerve to ulnar nerve transfer in severe compressive ulnar neuropathy.

Background: With advances in the surgical management for severe ulnar neuropathy with the introduction of the super charged-end-to-side (SETS) anterior interosseous nerve (AIN) to ulnar nerve transfer, a simple and reliable outcome measure is required. There is currently not "one" standardized outcome measure used to represent and compare results.

Purpose: To present the abduction hand diagram as a "novel", reproducible, and simple outcome measure for patients with severe ulnar neuropathy.

Radiation-induced recto-urinary fistula: A dreaded complication with devastating consequences.

Purpose: This study was undertaken to evaluate our 16-year experience with fistulas between the rectum and urethra or urinary bladder, collectively called recto-urinary fistulas (RUFs), and their devastating consequences in patients treated with radiation for prostate cancer.

Methods: We downloaded the records of all patients with radiation-related RUFS from 2004 to 2020 at our institution using the electronic medical record system. Details concerning patient demographics, clinical presentation, diagnostic approaches and surgical management were obtained and assessed.

Development of the data registry for the Cardiac Neurodevelopmental Outcome Collaborative.

Children with congenital heart disease (CHD) can face neurodevelopmental, psychological, and behavioural difficulties beginning in infancy and continuing through adulthood. Despite overall improvements in medical care and a growing focus on neurodevelopmental screening and evaluation in recent years, neurodevelopmental disabilities, delays, and deficits remain a concern. The Cardiac Neurodevelopmental Outcome Collaborative was founded in 2016 with the goal of improving neurodevelopmental outcomes for individuals with CHD and pediatric heart disease.

Single Ventricle Reconstruction III: Brain Connectome and Neurodevelopmental Outcomes: Design, Recruitment, and Technical Challenges of a Multicenter, Observational Neuroimaging Study.

Unlabelled: Patients with hypoplastic left heart syndrome who have been palliated with the Fontan procedure are at risk for adverse neurodevelopmental outcomes, lower quality of life, and reduced employability. We describe the methods (including quality assurance and quality control protocols) and challenges of a multi-center observational ancillary study, SVRIII (Single Ventricle Reconstruction Trial) Brain Connectome. Our original goal was to obtain advanced neuroimaging (Diffusion Tensor Imaging and Resting-BOLD) in 140 SVR III participants and 100 healthy controls for brain connectome analyses.

Developmental care pathway for hospitalised infants with CHD: on behalf of the Cardiac Newborn Neuroprotective Network, a Special Interest Group of the Cardiac Neurodevelopmental Outcome Collaborative.

Infants and children born with CHD are at significant risk for neurodevelopmental delays and abnormalities. Individualised developmental care is widely recognised as best practice to support early neurodevelopment for medically fragile infants born premature or requiring surgical intervention after birth. However, wide variability in clinical practice is consistently demonstrated in units caring for infants with CHD.

Developmental Care for Hospitalized Infants With Complex Congenital Heart Disease: A Science Advisory From the American Heart Association.

Developmental disorders, disabilities, and delays are a common outcome for individuals with complex congenital heart disease, yet targeting early factors influencing these conditions after birth and during the neonatal hospitalization for cardiac surgery remains a critical need. The purpose of this science advisory is to (1) describe the burden of developmental disorders, disabilities, and delays for infants with complex congenital heart disease, (2) define the potential health and neurodevelopmental benefits of developmental care for infants with complex congenital heart disease, and (3) identify critical gaps in research aimed at evaluating developmental care interventions to improve neurodevelopmental outcomes in complex congenital heart disease. This call to action targets research scientists, clinicians, policymakers, government agencies, advocacy groups, and health care organization leadership to support funding and hospital-based infrastructure for developmental care in the complex congenital heart disease population.

Palliative Care Across the Life Span for Children With Heart Disease: A Scientific Statement From the American Heart Association.

Aim: This summary from the American Heart Association provides guidance for the provision of primary and subspecialty palliative care in pediatric congenital and acquired heart disease.

Methods: A comprehensive literature search was conducted from January 2010 to December 2021. Seminal articles published before January 2010 were also included in the review.