Alpelisib for the treatment of PIK3CA-related head and neck lymphatic malformations and overgrowth.

Purpose: PIK3CA-related overgrowth spectrum (PROS) conditions of the head and neck are treatment challenges. Traditionally, these conditions require multiple invasive interventions, with incomplete malformation removal, disfigurement, and possible dysfunction. Use of the PI3K inhibitor alpelisib, previously shown to be effective in PROS, has not been reported in PIK3CA-associated head and neck lymphatic malformations (HNLMs) or facial infiltrating lipomatosis (FIL).

Periscopic technique in Norwood operation is associated with better preservation of early ventricular function.

Objective: Although the right ventricle (RV) to pulmonary artery conduit in stage 1 Norwood operation results in improved interstage survival, the long-term effects of the ventriculotomy used in the traditional technique remain a concern. The periscopic technique (PT) of RV to pulmonary artery conduit placement has been described as an alternative technique to minimize RV injury. A retrospective study was performed to compare the effects of traditional technique and PT on ventricular function following Norwood operation.

Computational fluid dynamics modeling aiding surgical planning in a toddler with Parkes Weber syndrome.

Parkes Weber syndrome is a fast-flow and slow-flow vascular anomaly with limb overgrowth that can lead to congestive heart failure and limb ischemia. Current management strategies have focused on symptom management with focal embolization. A pediatric case with early onset heart failure is reported.

Transplacental Respiratory Syncytial Virus and Influenza Virus Antibody Transfer in Alaska Native and Seattle Mother-Infant Pairs.

Background: Alaska Native (AN) infants are at risk for severe disease due to respiratory syncytial virus (RSV) and influenza. Maternal immunization protects young infants through transplacental antibody transfer. RSV- and influenza-specific transplacental antibody transfer in mother-infant pairs has not previously been evaluated in the AN population.

Exploring communication between parents and clinical teams following children's heart surgery: a survey in the UK.

Objective: To explore communication between clinicians and families of children undergoing heart surgery.

Design: This study was part of a larger study to select, define and measure the incidence of postoperative complications in children undergoing heart surgery. Parents of children recruited to a substudy between October 2015 and December 2017 were asked to complete a questionnaire about communication during their child's inpatient stay.

Clinical application of molecular genetics in lymphatic malformations.

Objectives: To describe the clinical presentation of lymphatic malformations (LM) and genotypically associated disorders and to summarize the recent literature regarding the genetic etiology of LM and provide a biologic correlation to medical and surgical management.

Results: LM are congenital lesions derived from a developmental abnormality of the lymphatic vessels. The severity of disease varies widely and complications can occur with higher staged disease and those associated with a known constellation of symptoms.

Ethnic-specific mortality of infants undergoing congenital heart surgery in England and Wales.

Purpose: To investigate ethnic differences in mortality for infants with congenital heart defects (CHDs) undergoing cardiac surgery or interventional catheterisation.

Design: Observational study of survival to age 1 year using linked records from routine national paediatric cardiac surgery and intensive care audits. Mortality risk was investigated using multivariable Poisson models with multiple imputation.

Medical Management of Vascular Anomalies.

Purpose Of Review: This chapter will summarize the most recent literature regarding the current state of medical treatment for vascular anomalies.

Recent Findings: Research into the biology of these anomalies has strengthened our understanding of each anomaly and has helped to pave the way for more tailored treatment options involving molecular and/or genetic targets.

Summary: While there is still a role for surgical intervention, medical therapies that target the etiology of vascular anomalies may represent an alternative or adjunctive approach in the management of these lesions.

Engaging police to identify challenging school crossings and potential improvements.

This paper discusses the value of police officers as vital sources of information regarding pedestrian safety in their communities by presenting results from a survey of traffic safety police officers. The survey requested information on school crossings that the officers considered most challenging for pedestrians. Officers specified the intersections or mid-block locations with school crossings, and answered questions about elements of the locations, such as what makes the locations challenging, and what might be done to improve conditions at these locations.

Issues facing families of infants discharged after cardiac surgery: the perceptions of charity helpline staff.

Aim: To elicit the perceptions of helpline staff who talk to parents of children discharged after cardiac surgery in infancy about parents' key concerns.

Methods: A qualitative study involving semistructured interviews with 10 staff at four heart charities. Interviews were recorded, transcribed and analysed using Framework analysis.

Interventional treatments and risk factors in patients born with hypoplastic left heart syndrome in England and Wales from 2000 to 2015.

Objective: To describe the long-term outcomes, treatment pathways and risk factors for patients diagnosed with hypoplastic left heart syndrome (HLHS) in England and Wales.

Methods: The UK's national audit database captures every procedure undertaken for congenital heart disease and updated life status for resident patients in England and Wales. Patients with HLHS born between 2000 and 2015 were identified using codes from the International Paediatric and Congenital Cardiac Code.

Combining qualitative and quantitative operational research methods to inform quality improvement in pathways that span multiple settings.

Background: Improving integration and continuity of care across sectors within resource constraints is a priority in many health systems. Qualitative operational research methods of problem structuring have been used to address quality improvement in services involving multiple sectors but not in combination with quantitative operational research methods that enable targeting of interventions according to patient risk. We aimed to combine these methods to augment and inform an improvement initiative concerning infants with congenital heart disease (CHD) whose complex care pathway spans multiple sectors.

Ethnic and socioeconomic variation in incidence of congenital heart defects.

Introduction: Ethnic differences in the birth prevalence of congenital heart defects (CHDs) have been reported; however, studies of the contemporary UK population are lacking. We investigated ethnic variations in incidence of serious CHDs requiring cardiac intervention before 1 year of age.

Methods: All infants who had a cardiac intervention in England and Wales between 1 January 2005 and 31 December 2010 were identified in the national congenital heart disease surgical audit and matched with paediatric intensive care admission records to create linked individual child records.

The Experience of Long-Stay Parents in the ICU: A Qualitative Study of Parent and Staff Perspectives.

Objective: Meeting the needs of parents of critically ill children is increasingly being recognized as an important factor in the child's clinical outcome as well as the implications it has for future parenting. Little is specifically known about the experience of parents who have a child in the ICU for a prolonged period. Our objective was to understand the experiences of this group to assist in the identification of mechanisms for providing support.

Self-Reported Health Experiences of Children Living with Congenital Heart Defects: Including Patient-Reported Outcomes in a National Cohort Study.

Background: Understanding children's views about living with congenital heart defects (CHDs) is fundamental to supporting their successful participation in daily life, school and peer relationships. As an adjunct to a health and quality of life outcomes questionnaire, we asked school-age children who survived infant heart procedures to describe their experiences of living with CHDs.

Methods: In a UK-wide cohort study, children aged 10 to 14 years with CHDs self-completed postal questionnaires that included an open question about having a 'heart problem'.

Identifying improvements to complex pathways: evidence synthesis and stakeholder engagement in infant congenital heart disease.

Objectives: Many infants die in the year following discharge from hospital after surgical or catheter intervention for congenital heart disease (3-5% of discharged infants). There is considerable variability in the provision of care and support in this period, and some families experience barriers to care. We aimed to identify ways to improve discharge and postdischarge care for this patient group.

The Challenges of Caring for Long-Stay Patients in the PICU.

Objective: Compared to shorter-stay patients, caring for long-stay patients in the ICU entails a disproportionate burden for staff. Our objective was to gain a deeper understanding of the impact on staff of caring for children who have a prolonged stay on the PICU.

Design: Qualitative study based on semi-structured interviews.

Death and Emergency Readmission of Infants Discharged After Interventions for Congenital Heart Disease: A National Study of 7643 Infants to Inform Service Improvement.

Background: Improvements in hospital-based care have reduced early mortality in congenital heart disease. Later adverse outcomes may be reducible by focusing on care at or after discharge. We aimed to identify risk factors for such events within 1 year of discharge after intervention in infancy and, separately, to identify subgroups that might benefit from different forms of intervention.

"I was so worried about every drop of milk" - feeding problems at home are a significant concern for parents after major heart surgery in infancy.

Increasing numbers of operations in small infants with complex congenital heart disease are being carried out in the UK year on year, with more surviving the initial operation. However, even after successful surgery some of these infants remain fragile when they are discharged home. The aim of the study was to elicit parents' experiences of caring for a child with complex needs after major congenital heart surgery.

Going home after infant cardiac surgery: a UK qualitative study.

Objective: To qualitatively assess the discharge processes and postdischarge care in the community for infants discharged after congenital heart interventions in the first year of life.

Design: Qualitative study using semistructured interviews and Framework Analysis.

Setting: UK specialist cardiac centres and the services their patients are discharged to.

Signs of deterioration in infants discharged home following congenital heart surgery in the first year of life: a qualitative study.

Aims: To describe the ways in which parents recognise and make decisions about their child's symptoms following discharge home after congenital heart interventions in the first year of life and their experiences of seeking help.

Methods: This was a qualitative study involving semistructured interviews with parents. Twenty-one parents were recruited to the study.

Performance monitoring of the arterial switch operation: a moving target.

Objectives: Twenty years ago our institution published an analysis of a cluster of failures associated with the arterial switch operation (ASO). The concept of 'near miss' was explored to detect warning signs of suboptimal performance. The aim of the current study was to review the parameters best suited to monitoring early ASO outcomes in the modern setting and re-examine the concept of near misses as failure equivalents.