In recent decades, survival of children with complex congenital heart disease has improved considerably. Specifically, children with a variety of congenital heart defects resulting in ‘single ventricle’ physiology can now undergo palliative surgery that allows survival beyond the neonatal period, and in many cases into adulthood, despite having a single functional ventricular pumping chamber supplying both the pulmonary and systemic circulation. Our growing understanding of the functionally univentricular heart has resulted in freedom from Fontan failure of >50% at 25 years post-Fontan. Yet there is still a fair amount of knowledge to be gained, specifically as it relates to the pulmonary circulation in this group of patients. Knowledge gaps relate not only to the pulmonary circulation after Fontan operation, but also at each stage of the single ventricle surgical palliation, including the native physiology prior to any intervention. The pulmonary circulation is affected by multiple issues related to the single ventricle, including specific details of the anatomy unique to each patient, any intervention(s) undertaken, and potential complications such as aortopulmonary collaterals, protein losing enteropathy, plastic bronchitis, venovenous collaterals, pulmonary arteriovenous fistulae, ventricular dysfunction, pulmonary venous stenosis, and more. This chapter will review the current knowledge with regard to the pulmonary circulation in the single ventricle patient, primarily after the Fontan operation. Additionally, it is our hope to help the practitioner assess the pulmonary circulation in the single ventricle patient; we will also discuss the evidence behind and approach to treatment strategies in order to optimize the pulmonary circulation in this complex group of patients.

Purpose of review: Sudden cardiac arrest and sudden cardiac death are less common in children and adolescents than in the adult population. The outcomes from sudden cardiac arrest are generally quite poor in all ages and some data suggest that they are worse in the child and adolescent age group. In addition, the incidence of bystander cardiopulmonary resuscitation (CPR) and automated external defibrillator (AED) use is generally quite low although it is somewhat variable across communities. This review has been written in order to review the data for pediatric bystander CPR and AED use as it relates to out-of-hospital cardiac arrest (OHCA) survival. The purpose of this article is also to review endeavors at CPR--AED education in the context of improving both community bystander CPR/AED interventions and OHCA survival. Finally, this review will attempt to suggest some potential educational interventions in order to increase both bystander CPR-AED use and OHCA survival in local communities. Recent findings: Findings from several recent studies suggest that the incidence of bystander CPR--AED intervention is relatively low and that OHCA survival is also quite low in most communities. Several studies also suggest that community educational efforts can increase bystander CPR--AED interventions. The increase in OHCA survival may be attributed to the community educational efforts, yet that increase in survival is relatively small. A prospective high-school study has shown that in this very specific environment, a high incidence of CPR--AED use can be achieved, and that as a result the OHCA survival can be relatively high. Summary: It would be beneficial to attempt to ensure that all schools have a CPR--AED program along with a group of individuals trained to do CPR and use an AED. Not only should all schools have a cardiac emergency response plan (CERP) but all schools should have CPR--AED programs and all students should learn CPR and AED use prior to graduation. This strategy will ensure that we will have a community of individuals who would perform CPR and use an AED in the community and that in so doing we could increase the incidence of bystander CPR/AED use and increase the OHCA survival rate.

Purpose of review The optimal approach to screening young people to decrease the risk of sudden death remains unknown. It deserves the passionate attention that researchers, clinicians and families have given it. The new data from January 2018 to July 2019 are reviewed here. Recent findings Cardiac findings associated with a risk of sudden death were reported in 0.4% of screened athletes. Well run programs continue report varying sensitivity for screening ECGs (between 86 and 100%). One major article reported a higher incidence of sudden death in young people than has been previously published (6.8/100 000 athletes). Summary The rate of important findings in sophisticated screening programs is approximately 0.4%, suggesting that this is near the population rate of detectable disease in most athletic groups. ECGs are unquestionably capable of detecting disease that can be missed by history and physical, but the performance characteristics of ECGs continue to vary from study to study. In addition, the underlying cost and infrastructure of ECG and echocardiographic screening remains unaddressed by the recent literature. A few small studies have started to look at alternative technology approaches to ECG screening. http://links.lww.com/HCO/A58

Recent experiences with lay public cardiopulmonary resuscitation and use of ambulatory external defibrillators have been demonstrated to improve survival from out-of-hospital cardiac arrests from an abysmal 10% or less to as high as 30%. Though rare, sudden cardiac arrest in presumably healthy young people have been highly publicised over the last 25 years and have motivated the institution of school- and community-based programmes that facilitate first-responder resuscitation, including defibrillation. These efforts often begin at a grass roots level (usually parent-inspired advocacy groups) and through collaborative activities involving the schools, local businesses, and local administrative agencies are now having a meaningful impact in selected communities. The current focus of these activities is on school- and sports venue-based ambulatory external defibrillator programmes and formal cardiopulmonary resuscitation education in high schools. The extension of these programmes to the level of state mandates has been slower, and even when enacted, public funding has usually not been approved.