Achieving excellence in paediatric cardiac care in resource limited and resource plentiful settings and building successful care networks across different countries.

Background: The delivery of paediatric cardiac care across the world occurs in settings with significant variability in available resources. Irrespective of the resources locally available, we must always strive to improve the quality of care we provide to our patients and simultaneously deliver such care in the most efficient and cost-effective manner. The development of cardiac networks is used widely to achieve these aims.

Touching Technology-Parents' Experiences of Remote Consultations for Children With Severe Congenital Cardiac Conditions: Quasi-Experimental Cohort Study.

Background: Remote consultations (RCs) using videoconferencing was recommended by the General Medical Council as the method for clinicians to provide patient consultations during the COVID-19 pandemic. Facilitating this while providing high-quality care depends on the usability and acceptability of the technology.

Objective: This project aimed to investigate parents' experiences of using videoconferencing technology for real-time RCs with children who had congenital heart defects during the COVID-19 pandemic lockdown.

Epicardial Pacemaker Causing Cardiac Strangulation.

Cardiac strangulation is a rare but potentially lethal complication of epicardial pacemaker insertion. We present the case of a 9-year-old girl who was identified as having cardiac strangulation on routine follow-up for an epicardial pacemaker inserted on day 1 of life for congenital complete heart block (CCHB). The potential clinical presentations and risk factors for pacemaker strangulation are then discussed.

Paediatric heart failure - understanding the pathophysiology and the current role of cardiac biomarkers in clinical practice.

Introduction: Paediatric heart failure is a common clinical syndrome that may be experienced by children with congenital heart disease (CHD) and/or cardiomyopathy. It is characterised by clinical signs/symptoms which reflect the underlying pathophysiology based on one of three main clinical states: Pulmonary over-circulation, pressure overload, and ventricular failure. Current diagnosis relies on clinical assessment and echocardiogram imaging as cardiac biomarkers has been predominantly scientific to date.

How to interpret cardiac biomarkers in children?

Cardiac biomarkers are used as first-line diagnostic tools in suspected myocardial injury and heart failure in adult patients. Their use in paediatric patients has been limited by variability caused by age, gender and the presence of an underlying congenital cardiac condition. There are established reference ranges for both NT-proBNP and troponin in healthy children, but these cannot be applied to all paediatric patients because of limited large studies focusing on children with congenital heart disease and/or cardiomyopathy.

Editorial Board Composition Among Pediatric Cardiology Journals: Time to Cast the Net Wider.

Background: No data currently exist on the diversity of editorial board members (EBMs) of pediatric cardiology journals.

Objectives: The objective was to investigate the editorial boards of 5 pediatric cardiology journals to assess the composition of these boards in terms of the geographical, gender, and economic representation of their members.

Methods: Information on EBMs was collected directly from 5 journal websites accessed in February 2022.

Prevalence and trends of congenital heart defects among live births from 2005 to 2014 in Northern Ireland.

Rationale: Congenital heart defects (CHD) are the most frequent group of congenital anomalies representing a significant burden of mortality and morbidity and health service load.

Objective: In the Northern Ireland population, served by a single paediatric cardiology centre, we determine the prevalence and trends of CHD among live births.

Methods: This is a descriptive cross-sectional population-based study, using the paediatric cardiology database.

MG132 Induces Progerin Clearance and Improves Disease Phenotypes in HGPS-like Patients' Cells.

Progeroid syndromes (PS), including Hutchinson-Gilford Progeria Syndrome (HGPS), are premature and accelerated aging diseases, characterized by clinical features mimicking physiological aging. Most classical HGPS patients carry a de novo point mutation within exon 11 of the gene encoding A-type lamins. This mutation activates a cryptic splice site, leading to the production of a truncated prelamin A, called prelamin A ∆50 or progerin, that accumulates in HGPS cell nuclei and is a hallmark of the disease.

Neurodevelopmental and psychosocial interventions for individuals with CHD: a research agenda and recommendations from the Cardiac Neurodevelopmental Outcome Collaborative.

In 2018, the Neurodevelopmental and Psychosocial Interventions Working Group of the Cardiac Neurodevelopmental Outcome Collaborative convened through support from an R13 grant from the National Heart, Lung, and Blood Institute to survey the state of neurodevelopmental and psychosocial intervention research in CHD and to propose a slate of critical questions and investigations required to improve outcomes for this growing population of survivors and their families. Prior research, although limited, suggests that individualised developmental care interventions delivered early in life are beneficial for improving a range of outcomes including feeding, motor and cognitive development, and physiological regulation. Interventions to address self-regulatory, cognitive, and social-emotional challenges have shown promise in other medical populations, yet their applicability and effectiveness for use in individuals with CHD have not been examined.

Exercise prescription improves exercise tolerance in young children with CHD: a randomised clinical trial.

Objective: The main objective of this study was to ascertain if a structured intervention programme can improve the biophysical health of young children with congenital heart disease (CHD). The primary end point was an increase in measureable physical activity levels following the intervention.

Methods: Patients aged 5-10 years with CHD were identified and invited to participate.

Risk factors for congenital heart disease: The Baby Hearts Study, a population-based case-control study.

We investigated the role of maternal environmental factors in the aetiology of congenital heart disease (CHD). A population-based case-control study (242 CHD cases, 966 controls) was conducted using an iPad questionnaire for mother with linkage to maternity and first trimester prescription records. Risk of CHD was associated with low maternal education (OR adjusted for confounders 1.

Neurodevelopmental Outcomes Among Children With Congenital Heart Disease: At-Risk Populations and Modifiable Risk Factors.

As survivable outcomes among patients with complex congenital heart disease (cCHD) have continued to improve over the last several decades, more attention is being dedicated to interventions that impact not just survival but quality of life among patients with cCHD. In particular, patients with cCHD are at risk for impaired neurodevelopmental outcomes. In this review summarizing select presentations given at the 14th Annual Pediatric Cardiac Intensive Care Society's Annual Meeting in 2019, we discuss the neurodevelopmental phenotype of patients with cCHD, patients at greatest risk of impaired development, and three specific modifiable risk factors impacting development.

The CHIP-Family study to improve the psychosocial wellbeing of young children with congenital heart disease and their families: design of a randomized controlled trial.

Background: Children with congenital heart disease (CHD) are at increased risk for behavioral, emotional, and cognitive problems. They often have reduced exercise capacity and participate less in sports, which is associated with a lower quality of life. Starting school may present more challenges for children with CHD and their families than for families with healthy children.

Multidisciplinary family-centred psychosocial care for patients with CHD: consensus recommendations from the AEPC Psychosocial Working Group.

Because of the enormous advances in the medical treatment of CHD, the long-term survival of patients suffering from this disease has increased significantly. Currently, about 90% of patients reach adulthood, which entails many new challenges both for patients and their families and for healthcare professionals. The main objective of family-centred psychosocial care is to strengthen the emotional resilience of chronically ill patients and their families by adopting a holistic approach.

Physical and occupational therapy utilization in a pediatric intensive care unit.

Purpose: To characterize the use of physical therapy (PT) and occupational therapy (OT) consultation in our pediatric intensive care unit (PICU).

Materials And Methods: We studied children aged 1week-18years admitted to a tertiary care PICU for ≥3days. Patient characteristics, details of PT and OT sessions and adverse events were collected.

Antisense-Based Progerin Downregulation in HGPS-Like Patients' Cells.

Progeroid laminopathies, including Hutchinson-Gilford Progeria Syndrome (HGPS, OMIM #176670), are premature and accelerated aging diseases caused by defects in nuclear A-type Lamins. Most HGPS patients carry a de novo point mutation within exon 11 of the LMNA gene encoding A-type Lamins. This mutation activates a cryptic splice site leading to the deletion of 50 amino acids at its carboxy-terminal domain, resulting in a truncated and permanently farnesylated Prelamin A called Prelamin A Δ50 or Progerin.

Survey of paediatricians' opinions on a regional paediatric telecardiology service.

Aim: The aim of this study is to evaluate consultant general paediatricians' opinions of a UK paediatric telecardiology service.

Methods: A structured questionnaire was developed and sent to all consultant paediatricians working in a district general hospital in Northern Ireland.

Results: Paediatricians (n = 35) regarded the regional paediatric telecardiology service as very useful and of good value for money.

Postoperative pulmonary calcification in a child with truncus arteriosus.

Pulmonary calcification is uncommon in children. It is rarely described following cardiac surgery. Here, the authors describe the case of an infant who developed extensive pulmonary calcification following the repair of truncus arteriosus.

A sibling-controlled, prospective study of outcomes at home and school in children with severe congenital heart disease.

Objectives: The objectives of this study were to compare behaviour problems and competencies, at home and school, in 7-year-old children with congenital heart disease with a sibling control group, to examine the prospective determinants of outcome from infancy, and to explore whether any gains were maintained in our sub-group of children who had participated in a previous trial of psychological interventions in infancy.

Methods: A total of 40 children who had undergone surgery to correct or palliate a significant congenital heart defect in infancy were compared (Child Behavior Checklist) with a nearest-age sibling control group (18 participants). Comparisons were made between sub-groups of children and families who had and had not participated in an early intervention trial.

A randomized controlled trial of interventions to promote adjustment in children with congenital heart disease entering school and their families.

Objective: To report on a randomized controlled trial of psychological interventions to promote adjustment in children with congenital heart disease and their families.

Method: Following baseline assessment, 90 children (aged 4-5 years) and their families were randomly assigned to an Intervention or Control group before entering school. 68 (76%) were retained at 10-month follow-up.