Bradley S. Marino

Part 13: Pediatric Basic Life Support 2010 American Heart Association Guidelines for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care

In contrast to adults, cardiac arrest in infants and children does not usually result from a primary cardiac cause. More often it is the terminal result of progressive respiratory failure or shock, also called an asphyxial arrest. Asphyxia begins with a variable period of systemic hypoxemia, hypercapnea, and acidosis, progresses to bradycardia and hypotension, and culminates with cardiac arrest.1 Another mechanism of cardiac arrest, ventricular fibrillation (VF) or pulseless ventricular tachycardia (VT), is the initial cardiac rhythm in approximately 5% to 15% of pediatric in-hospital and out-of-hospital cardiac arrests;2,–,9 it is reported in up to 27% of pediatric in-hospital arrests at some point during the resuscitation.6 The incidence of VF/pulseless VT cardiac arrest rises with age.2,4 Increasing evidence suggests that sudden unexpected death in young people can be associated with genetic abnormalities in myocyte ion channels resulting in abnormalities in ion flow (see “Sudden Unexplained Deaths,” below). Since 2010 marks the 50th anniversary of the introduction of cardiopulmonary resuscitation (CPR),10 it seems appropriate to review the progressive improvement in outcome of pediatric resuscitation from cardiac arrest. Survival from in-hospital cardiac arrest in infants and children in the 1980s was around 9%.11,12 Approximately 20 years later, that figure had increased to 17%,13,14 and by 2006, to 27%.15,–,17 In contrast to those favorable results from in-hospital cardiac arrest, overall survival to discharge from out-of-hospital cardiac arrest in infants and children has not changed substantially in 20 years and remains at about 6% (3% for infants and 9% for children and adolescents).7,9 It is unclear why the improvement in outcome from in-hospital cardiac arrest has occurred, although earlier recognition and management of at-risk patients on general inpatient units …

Neurodevelopmental Outcomes in Children With Congenital Heart Disease: Evaluation and Management A Scientific Statement From the American Heart Association

Background— The goal of this statement was to review the available literature on surveillance, screening, evaluation, and management strategies and put forward a scientific statement that would comprehensively review the literature and create recommendations to optimize neurodevelopmental outcome in the pediatric congenital heart disease (CHD) population. Methods and Results— A writing group appointed by the American Heart Association and American Academy of Pediatrics reviewed the available literature addressing developmental disorder and disability and developmental delay in the CHD population, with specific attention given to surveillance, screening, evaluation, and management strategies. MEDLINE and Google Scholar database searches from 1966 to 2011 were performed for English-language articles cross-referencing CHD with pertinent search terms. The reference lists of identified articles were also searched. The American College of Cardiology/American Heart Association classification of recommendations and levels of evidence for practice guidelines were used. A management algorithm was devised that stratified children with CHD on the basis of established risk factors. For those deemed to be at high risk for developmental disorder or disabilities or for developmental delay, formal, periodic developmental and medical evaluations are recommended. A CHD algorithm for surveillance, screening, evaluation, reevaluation, and management of developmental disorder or disability has been constructed to serve as a supplement to the 2006 American Academy of Pediatrics statement on developmental surveillance and screening. The proposed algorithm is designed to be carried out within the context of the medical home. This scientific statement is meant for medical providers within the medical home who care for patients with CHD. Conclusions— Children with CHD are at increased risk of developmental disorder or disabilities or developmental delay. Periodic developmental surveillance, screening, evaluation, and reevaluation throughout childhood may enhance identification of significant deficits, allowing for appropriate therapies and education to enhance later academic, behavioral, psychosocial, and adaptive functioning.

Pediatric Advanced Life Support: 2010 American Heart Association Guidelines for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care

For best survival and quality of life, pediatric basic life support (BLS) should be part of a community effort that includes prevention, early cardiopulmonary resuscitation (CPR), prompt access to the emergency response system, and rapid pediatric advanced life support (PALS), followed by integrated post–cardiac arrest care. These 5 links form the American Heart Association (AHA) pediatric Chain of Survival (Figure 1), the first 3 links of which constitute pediatric BLS. FIGURE 1. Pediatric Chain of Survival. Rapid and effective bystander CPR can be associated with successful return of spontaneous circulation (ROSC) and neurologically intact survival in children following out-of-hospital cardiac arrest.1,–,3 Bystander resuscitation may have the greatest impact for out-of-hospital respiratory arrest,4 because survival rates >70% have been reported with good neurologic outcome.5,6 Bystander resuscitation may also have substantial impact on survival from primary ventricular fibrillation (VF), because survival rates of 20% to 30% have been documented in children with sudden out-of-hospital witnessed VF.7 Overall about 6%8 of children who suffer an out-of-hospital cardiac arrest and 8% of those who receive prehospital emergency response resuscitation survive, but many suffer serious permanent brain injury as a result of their arrest.7,9,–,14 Out-of-hospital survival rates and neurological outcome can be improved with prompt bystander CPR,3,6,15,–,17 but only about one third to one half of infants and children who suffer cardiac arrest receive bystander CPR.3,9,12,18 Infants are less likely to survive out-of-hospital cardiac arrest (4%) than children (10%) or adolescents (13%), presumably because many infants included in the arrest figure are found dead after a substantial period of time, most from sudden infant death syndrome (SIDS).8 As in adults, survival is …

Impact of Oral Sildenafil on Exercise Performance in Children and Young Adults After the Fontan Operation A Randomized, Double-Blind, Placebo-Controlled, Crossover Trial

Background— Children and young adults with single-ventricle physiology have abnormal exercise capacity after the Fontan operation. A medication capable of decreasing pulmonary vascular resistance should allow improved cardiac filling and improved exercise capacity. Methods and Results— This study was a double-blind, placebo-controlled, crossover trial conducted in children and young adults after Fontan. Subjects were randomized to receive placebo or sildenafil (20 mg three times daily) for 6 weeks. After a 6-week washout, subjects crossed over for an additional 6 weeks. Each subject underwent an exercise stress test at the start and finish of each phase. After taking sildenafil, subjects had a significantly decreased respiratory rate and decreased minute ventilation at peak exercise. At the anaerobic threshold, subjects had significantly decreased ventilatory equivalents of carbon dioxide. There was no change in oxygen consumption during peak exercise, although there was a suggestion of improved oxygen consumption at the anaerobic threshold. Improvement at the anaerobic threshold was limited to the subgroup with single left or mixed ventricular morphology and to the subgroup with baseline serum brain natriuretic peptide levels ≥100 pg/mL. Conclusions— In this cohort, sildenafil significantly improved ventilatory efficiency during peak and submaximal exercise. There was also a suggestion of improved oxygen consumption at the anaerobic threshold in 2 subgroups. These findings suggest that sildenafil may be an important agent for improving exercise performance in children and young adults with single-ventricle physiology after the Fontan operation. Clinical Trial Registration— URL: . Unique identifier: [NCT00507819][1]. # Clinical Perspective {#article-title-31} [1]: /lookup/external-ref?link_type=CLINTRIALGOV&access_num=NCT00507819&atom=%2Fcirculationaha%2F123%2F11%2F1185.atomBackground— Children and young adults with single-ventricle physiology have abnormal exercise capacity after the Fontan operation. A medication capable of decreasing pulmonary vascular resistance should allow improved cardiac filling and improved exercise capacity. Methods and Results— This study was a double-blind, placebo-controlled, crossover trial conducted in children and young adults after Fontan. Subjects were randomized to receive placebo or sildenafil (20 mg three times daily) for 6 weeks. After a 6-week washout, subjects crossed over for an additional 6 weeks. Each subject underwent an exercise stress test at the start and finish of each phase. After taking sildenafil, subjects had a significantly decreased respiratory rate and decreased minute ventilation at peak exercise. At the anaerobic threshold, subjects had significantly decreased ventilatory equivalents of carbon dioxide. There was no change in oxygen consumption during peak exercise, although there was a suggestion of improved oxygen consumption at the anaerobic threshold. Improvement at the anaerobic threshold was limited to the subgroup with single left or mixed ventricular morphology and to the subgroup with baseline serum brain natriuretic peptide levels ≥100 pg/mL. Conclusions— In this cohort, sildenafil significantly improved ventilatory efficiency during peak and submaximal exercise. There was also a suggestion of improved oxygen consumption at the anaerobic threshold in 2 subgroups. These findings suggest that sildenafil may be an important agent for improving exercise performance in children and young adults with single-ventricle physiology after the Fontan operation. Clinical Trial Registration— URL: http://clinicaltrials.gov. Unique identifier: NCT00507819.

Risk factors for interstage death after stage 1 reconstruction of hypoplastic left heart syndrome and variants

OBJECTIVE The risk of death during the interstage period remains high after stage 1 reconstruction for single ventricle lesions, despite improved surgical results. The purpose of this study is to identify risk factors for interstage death and to describe the events leading to interstage death. METHODS A nested case-control study was conducted of 368 patients who underwent stage 1 reconstruction at a single center between January 1998 and April 2005. RESULTS Among the 313 (85%) hospital survivors, there were 33 (10.5%) interstage deaths. Cases more frequently presented with intact or restrictive atrial septum (9 [27%] vs 4 [4%]; P < .001), were older at the time of surgery (5 [2-40] vs 3 [1-42] days; P = .005), had more postoperative arrhythmias (12 [36%] vs 15 [15%]; P = .01), and a higher incidence of airway or respiratory complications (12 [36%] vs 19 [19%]; P = .04). By multivariate analysis, only intact atrial septum (odds ratio 7.6; 95% confidence intervals 1.9-29.6; P = .003) and age at operation greater than 7 days (odds ratio 3.8; 95% confidence intervals 1.3-11.2; P = .017) were predictors of interstage death. CONCLUSIONS The presence of intact atrial septum and older age at the time of surgery are associated with a higher risk of interstage death. In addition, postoperative arrhythmia and airway complications are associated with a higher risk of interstage death in univariate analysis. The results of this study provide a focus for interstage monitoring and risk stratification of these high-risk infants, which may improve overall survival.

Obesity Is a Common Comorbidity in Children With Congenital and Acquired Heart Disease

OBJECTIVES. Obesity may pose additional cardiovascular risk to children with acquired and congenital heart disease. Many children with heart disease are sedentary as a result of physician-, parent-, and/or self-imposed restrictions. The aim of this study was to evaluate the impact of the epidemic of obesity on children with heart disease. PATIENTS AND METHODS. A cross-sectional review was performed of children evaluated in 2004 at 2 cardiology outpatient clinics. Differences in the prevalence of obese (BMI ≥ 95%) and overweight (BMI 85%–95%) children were compared with national data and healthy control subjects. Dictated letters were reviewed to determine whether obesity was discussed with referring practitioners. RESULTS. Of 2921 patients assessed, 1523 had heart disease. Diagnostic subgroups included “mild” heart disease (n = 401), arrhythmia (n = 447), biventricular repair (n = 511), univentricular palliation (Fontan; n = 108), and heart transplantation (n = 56). More than 25% of the patients with heart disease were obese or overweight; the prevalence of obese and overweight children was significantly lower only in the Fontan group (15.9%). Pediatric cardiologists failed to document obesity or weight counseling in the majority of clinic letters. CONCLUSIONS. Obesity is common in children with congenital and acquired heart disease. Pediatric cardiologists demonstrate inadequate communication regarding this problem to referring practitioners. Healthy-lifestyle counseling and routine exercise in children with heart disease may be underemphasized.

Outcomes after the Fontan procedure

Over the past two decades, advances in congenital heart surgery, pediatric cardiology, and intensive care medicine have dramatically increased the survival of infants with critical congenital heart disease. The group of patients that has perhaps benefited the most from this progress has been the single-ventricle population. Staged palliation culminating in the Fontan procedure has resulted in a decreasing mortality rate and an increase in the number of single-ventricle survivors. Over the past 18 months, many studies have focused on outcomes after the Fontan procedure. These reports demonstrate progressive improvement in early postoperative survival and intermediate and late postoperative outcomes due to surgical innovations, such as the lateral tunnel and extracardiac Fontan modifications, and fenestration, as well as technological improvements, such as modified ultrafiltration. Despite these improvements, significant morbidity remains after the Fontan completion, including myocardial systolic and diastolic dysfunction, systemic arterial and venous hemodynamic abnormalities, diminished exercise capacity, arrhythmias, protein-losing enteropathy, somatic growth retardation, neo-aortic valve root dilation and insufficiency, thromboembolic complications, and below-average cognitive development.

Lower weight-for-age z score adversely affects hospital length of stay after the bidirectional Glenn procedure in 100 infants with a single ventricle

OBJECTIVE Poor growth has been described in infants with a single ventricle; however, little is known regarding its effect on surgical outcomes. We sought to assess the effect of nutritional status at the time of the bidirectional Glenn procedure on short-term outcomes. METHODS We performed a retrospective case series of children who underwent the bidirectional Glenn procedure at our institution between January 2001 and December 2007. Anthropometric measurements were recorded at the time of neonatal admission and the bidirectional Glenn procedure. Data from preoperative echocardiograms and cardiac catheterization were recorded. The primary outcome variable was length of hospital stay. RESULTS Data on 100 infants were included for analysis. Age at the time of the bidirectional Glenn procedure was 5.1 months (range, 2.4-10 months). The median weight-for-age z score at birth was -0.4 (range, -2.6 to 3.2), and by the time of the bidirectional Glenn procedure, it had decreased to -1.3 (range, -3.9 to 0.6). In multivariable modeling longer postoperative hospital stays were predicted by lower weight-for-age z score (P = .02), younger age (P < .001), being fed through a gastrostomy tube (P = .01), and undergoing concomitant aortic arch reconstruction (P < .001) at the time of the bidirectional Glenn procedure. CONCLUSIONS There is suboptimal weight gain between neonatal discharge and the bidirectional Glenn procedure. A lower weight-for-age z score and younger age at the time of the bidirectional Glenn procedure affects length of hospital stay independent of hemodynamic or echocardiographic variables.

Aortic morphometry and microcephaly in hypoplastic left heart syndrome

Microcephaly is a marker of abnormal fetal cerebral development, and a known risk factor for cognitive dysfunction. Patients with hypoplastic left heart syndrome have been found to have an increased incidence of abnormal neurodevelopmental outcomes. We hypothesized that reduced cerebral blood flow from the diminutive ascending aorta and transverse aortic arch in the setting of hypoplastic left heart syndrome may influence fetal growth of the brain. The purpose of our study, therefore, was to define the prevalence of microcephaly in full-term infants with hypoplastic left heart syndrome, and to investigate potential cardiac risk factors for microcephaly. We carried out a retrospective review of full-term neonates with hypoplastic left heart syndrome. Eligible patients had documented indexes of birth weight, and measurements of length, and head circumference, as well as adequate echocardiographic images for measurement of the diameters of the ascending aorta and transverse aortic arch. We used logistic regression for analysis of the data. A total of 129 neonates met the criteria for inclusion, with 15 (12%) proving to have microcephaly. The sizes of their heads were disproportionately smaller than their weights (p less than 0.001) and lengths (p less than 0.001) at birth. Microcephaly was associated with lower birth weight (p less than 0.001), lower birth length (p equal to 0.007), and a smaller diameter of the ascending aorta (p equal to 0.034), but not a smaller transverse aortic arch (p equal to 0.619), or aortic atresia (p equal to 0.969). We conclude that microcephaly was common in this cohort of neonates with hypoplastic left heart syndrome, with the size of the head being disproportionately smaller than weight and length at birth. Microcephaly was associated with a small ascending aorta, but not a small transverse aortic arch. Impairment of somatic growth may be an additional factor in the development of microcephaly in these neonates.

Part 6: Pediatric basic life support and pediatric advanced life support. 2015 International Consensus on Cardiopulmonary Resuscitation and Emergency Cardiovascular Care Science with Treatment Recommendations

The Pediatric Task Force reviewed all questions submitted by the International Liaison Committee on Resuscitation (ILCOR) member councils in 2010, reviewed all council training materials and resuscitation guidelines and algorithms, and conferred on recent areas of interest and controversy. We identified a few areas where there were key differences in council-specific guidelines based on historical recommendations, such as the A-B-C (Airway, Breathing, Circulation) versus C-A-B (Circulation, Airway, Breathing) sequence of provision of cardiopulmonary resuscitation (CPR), initial back blows versus abdominal thrusts for foreign-body airway obstruction, an upper limit for recommended chest compression rate, and initial defibrillation dose for shockable rhythms (2 versus 4 J/kg). We produced a working list of prioritized questions and topics, which was adjusted with the advent of new research evidence. This led to a prioritized palate of 21 PICO (population, intervention, comparator, outcome) questions for ILCOR task force focus. The 2015 process was supported by information specialists who performed in-depth systematic searches, liaising with pediatric content experts so that the most appropriate terms and outcomes and the most relevant publications were identified. Relevant adult literature was considered (extrapolated) in those PICO questions that overlapped with other task forces, or when there were insufficient pediatric data. In rare circumstances (in the absence of sufficient human data), appropriate animal studies were incorporated into reviews of the literature. However, these data were considered only when higher levels of evidence were not available and the topic was deemed critical. When formulating the PICO questions, the task force felt it important to evaluate patient outcomes that extend beyond return of spontaneous circulation (ROSC) or discharge from the pediatric intensive care unit (PICU). In recognition that the measures must have meaning, not only to clinicians but also to parents and caregivers, longer-term outcomes at 30 …