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In summary, the evidence review process has attempted to provide a systematic review of the scientific literature using a priori defined methods. The details and steps of the literature review are transparent and replicable. External opinions and community critique are highly valued, and the final products represent the combined labor of hundreds of participants.

The recommendations for electrical therapies described in this section are designed to improve survival from SCA and life-threatening arrhythmias. Whenever defibrillation is attempted, rescuers must coordinate high-quality CPR with defibrillation to minimize interruptions in chest compressions and to ensure immediate resumption of chest compressions after shock delivery. The high first-shock efficacy of newer biphasic defibrillators led to the recommendation of single shocks plus immediate CPR instead of 3-shock sequences that were recommended prior to 2005 to treat VF. Further data are needed to refine recommendations for energy levels for defibrillation and cardioversion using biphasic waveforms.

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 …

The 2010 ILCOR Pediatric Task Force experts developed 55 questions related to pediatric resuscitation. Topics were selected based on the 2005 Consensus on Science and Treatment Recommendations (CoSTR) document,1,2 emerging science, and newly identified issues. Not every topic reviewed for the 2005 International Consensus on Science was reviewed in the 2010 evidence evaluation process. In general, evidence-based worksheets were assigned to at least 2 authors for each topic. The literature search strategy was first reviewed by a “worksheet expert” for completeness. The expert also approved the final worksheet to ensure that the levels of evidence were correctly assigned according to the established criteria. Worksheet authors were requested to draft CoSTR statements (see Part 3: Evidence Evaluation Process). Each worksheet author or pair of authors presented their topic to the Task Force in person or via a webinar conference, and Task Force members discussed the available science and revised the CoSTR draft accordingly. These draft CoSTR summaries were recirculated to the International Liaison Committee on Resuscitation (ILCOR) Pediatric Task Force for further refinement until consensus was reached. Selected controversial and critical topics were presented at the 2010 ILCOR International Evidence Evaluation conference in Dallas, Texas, for further discussion to obtain additional input and feedback. This document presents the 2010 international consensus on the science, treatment, and knowledge gaps for each pediatric question. The most important changes or points of emphasis in the recommendations for pediatric resuscitation since the publication of the 2005 ILCOR International Consensus on CPR and ECC Science With Treatment Recommendations1,2 are summarized in the following list. The scientific evidence supporting these changes is detailed in this document. Additional evidence shows that healthcare providers do not reliably determine the presence or absence of a pulse in infants or children. New evidence documents the important role of ventilations in CPR for infants and children. However, rescuers who are unable or unwilling to provide ventilations should be encouraged to perform compression-only CPR. To achieve effective chest compressions, rescuers should compress at least one third the anterior-posterior dimension of the chest. This corresponds to approximately 1½ inches (4 cm) in most infants and 2 inches (5 cm) in most children. When shocks are indicated for ventricular fibrillation (VF) or pulseless ventricular tachycardia (VT) in infants and children, an initial energy dose of 2 to 4 J/kg is reasonable; doses higher than 4 J/kg, especially if delivered with a biphasic defibrillator, may be safe and effective. More data support the safety and effectiveness of cuffed tracheal tubes in infants and young children, and the formula for selecting the appropriately sized cuffed tube was updated. The safety and value of using cricoid pressure during emergency intubation are not clear. Therefore, the application of cricoid pressure should be modified or discontinued if it impedes ventilation or the speed or ease of intubation. Monitoring capnography/capnometry is recommended to confirm proper endotracheal tube position. Monitoring capnography/capnometry may be helpful during CPR to help assess and optimize quality of chest compressions. On the basis of increasing evidence of potential harm from exposure to high-concentration oxygen after cardiac arrest, once spontaneous circulation is restored, inspired oxygen concentration should be titrated to limit the risk of hyperoxemia. Use of a rapid response system in a pediatric inpatient setting may be beneficial to reduce rates of cardiac and respiratory arrest and in-hospital mortality. Use of a bundled approach to management of pediatric septic shock is recommended. The young victim of a sudden, unexpected cardiac arrest should have an unrestricted, complete autopsy, if possible, with special attention to the possibility of an underlying condition that predisposes to a fatal arrhythmia. Appropriate preservation and genetic analysis of tissue should be considered; detailed testing may reveal an inherited “channelopathy” that may also be present in surviving family members.

This report demonstrates variable clinical courses in several members of a family with tropomyosin-mediated hypertrophic cardiomyopathy (HCM) (L185R mutation). The index case was an 8-year-old girl who died from sudden cardiac death and was diagnosed with HCM on autopsy. Her father had minimal hypertrophy but had an implantable cardioverter defibrillator placed prophylactically with no appropriate shocks. Two brothers progressed from normal phenotype to HCM on follow-up, the younger with significant hypertrophy and the older with mild hypertrophy. They both had malignant arrhythmia courses with VF, which was terminated by ICD shock. In conclusion, family members with same genotype can have significantly variable phenotypes.

Defibrillation is the only effective treatment for ventricular fibrillation (VF). Optimal methods for defibrillation in children are derived and extrapolated from adult data. VF occurs as the initial rhythm in 8-20% of pediatric cardiac arrests. This has fostered a new interest in determining the optimal technique for pediatric defibrillation. This review will provide a brief background of the history of defibrillation and a review of the current literature on pediatric defibrillation. The literature search was performed through PubMed, using the MeSH headings of cardiopulmonary resuscitation, defibrillation and electric countershock. The authors' personal bibliographic files were also searched. Only published articles were chosen. The recommended energy dose has been 2 J/kg for 30 years, but recent reports may indicate that higher dosages may be more effective and safe. In 2005, the European Resuscitation Council recommended 4 J/kg as the initial dose, without escalation for subsequent shocks. Automated external defibrillators are increasingly used for pediatric cardiac arrest, and available reports indicate high success rates. Additional research on pediatric defibrillation is critical in order to be able to provide an equivalent standard of care for children in cardiac arrest and improve outcomes.

The validity of using adult physiologic criteria to triage injured children in the out-of-hospital setting remains unproven. Among children meeting adult field physiologic criteria, we assessed the availability of physiologic information, the incidence of death or prolonged hospitalization, and whether age-specific criteria would improve the specificity of the physiologic triage step.We analyzed a prospective, out-of-hospital cohort of injured children agedor =14 years collected from December 2005 through February 2007 by 237 emergency medical services (EMS) agencies transporting to 207 acute care hospitals (trauma and nontrauma centers) in 11 sites across the United States and Canada. Inclusion criteria were standard adult physiologic values: systolic blood pressure (SBP)or =90 mmHg, respiratory rate10 or29 breaths/min, Glasgow Coma Scale (GCS) scoreor =12, and field intubation attempt. Seven physiologic variables (including age-specific values) and three demographic and mechanism variables were included in the analysis. "High-risk" children included those who died (field or in-hospital) or were hospitalized2 days. The decision tree was derived and validated using binary recursive partitioning.Nine hundred fifty-five children were included in the analysis, of whom 62 (6.5%) died and 117 (12.3%) were hospitalized2 days. Missing values were common, ranging from 6% (respiratory rate) to 53% (pulse oximetry), and were associated with younger age and high-risk outcome. The final decision rule included four variables (assisted ventilation, GCS score11, pulse oximetry95%, and SBP96 mmHg), which demonstrated improved specificity (71.7% [95% confidence interval (CI) 66.7-76.6%]) at the expense of missing high-risk children (sensitivity 76.5% [95% CI 66.4-86.6%]).The incidence of high-risk injured children meeting adult physiologic criteria is relatively low and the findings from this sample do not support using age-specific values to better identify such children. However, the amount and pattern of missing data may compromise the value and practical use of field physiologic information in pediatric triage.

Public access defibrillation (PAD) programs are a major goal of the American Heart Association (AHA) to ensure that automated external defibrillators and trained lay rescuers are available in public areas where sudden cardiac arrest (SCA) is likely to occur. The Johnson County Early Defibrillation Task Force (JCEDTF) is a volunteer organization which distributed AEDs throughout Johnson County, Iowa. JCEDTF was responsible for initial training but ongoing support was the responsibility of each site.The purpose of this study was to evaluate compliance of community PAD sites to recommendations for site maintenance as proposed by the American Heart Association (AHA).Thirty-two surveys were distributed to community PAD sites that received assistance from JCEDTF. PAD sites were categorized into business, educational, or community sites. A twenty-five point scoring system to assess PDA programs was developed based on AHA recommendations. On-site evaluations were conducted to verify survey results and assess barriers to an effective PAD site. Differences among the three categories were measured with ANOVA.No site was able to comply with all the AHA guidelines for a PAD site. The mean score among all sites was 57% of possible points with no significant differences among the three categories. Business sites were more compliant with ongoing training compared to educational and community sites (p0.022).Community PAD sites in Johnson County currently do not comply with the recommendations for effective PAD sites. After initial training and establishment of community PAD sites, better methods for assuring ongoing training and maintenance are needed for sites to be effective.

Introduction: Recalls of implantable cardioverter defibrillator (ICD) generators have affected many patients. No information is available regarding the impact specifically on pediatric and congenital heart disease (CHD) patients. This study was undertaken to determine implications of ICD manufacturers’ advisories and recalls on children and CHD patients. Methods: The first part of this study involved single-center review of patients who underwent ICD placement between 2000 and 2005. Patients with ICDs affected by the 2005 advisories/recalls were reviewed for incidence of explantation, malfunction and complications. Secondly, members of the Pediatric and Congenital Electrophysiology Society (PACES) were queried for patients with affected devices, incidences of explantation, malfunction, and explant-related complications. Data were pooled for aggregate summary. Results: Among 233 patients who underwent ICD implantation at our institution during the study period, 58 (25%) patients had advisory/recalled devices and 13 of 58 (22%) underwent explantation following 3.1 ± 1.3 years implant duration. No defects were identified by the manufacturer. No patients experienced complications requiring reintervention or rehospitalization. Questionnaire responses were received from 22 PACES institutions, included 177 patients with affected devices, of which 76 (43%) were removed. One patient died from complications following revision, and 1 patient had complications requiring reoperation. Two explanted devices had loose headers; no other defects were discovered. Taken together, 2 of 89 explanted devices were defective, and 2 complications occurred from explantation. Conclusions: Advisories and recalls affect substantial numbers of pediatric and CHD patients. A significant proportion underwent explantation. Although complications are infrequent, there are important medical, psychosocial, and financial impacts associated with ICD replacement.

Injury is a major public health problem generating substantial morbidity, mortality, and economic burden on society. The majority of seriously injured persons are initially evaluated and cared for by prehospital providers, however the effect of emergency medical services (EMS) systems, EMS clinical care, and EMS interventions on trauma patient outcomes is largely unknown. Outcome-based information to guide future EMS care has been hampered by the lack of comprehensive, standardized, multi-center prehospital data resources that include meaningful patient outcomes. In this paper, we describe the background, design, development, implementation, content, and potential uses of the first North American comprehensive epidemiologic prehospital data registry for injured persons. This data registry samples patients from 264 EMS agencies transporting to 287 acute care hospitals in both the United States and Canada.

BACKGROUND . Ventricular fibrillation occurs in 10 –20% of pediatric cardiac arrests. Survival rates in children with ventricular fibrillation can be as high as 30% when the rhythm is identified and treated promptly. In the last five years, recommendations have been made for the use of automated external defibrillators(AED) in children 1– 8 years of age. OBJECTIVE . The goal of this study was to determine the awareness of American Heart Association (AHA) guidelines and statewide protocols concerning AED use in children ages 1– 8 among emergency medical providers after new guideline release. Availability of pediatric capable AED equipment was also assessed. METHODS . Surveys were distributed to EMS providers in Iowa and Montana within one year of the AHA advisory statement in 2003 recommending use of AEDs in children ages 1– 8, and again approximately one year after the 2005 AHA guidelines on cardiopulmonary resuscitation were published. In Iowa, there were concentrated efforts to disseminate information about AED use in children, while there were minimal efforts in Montana. RESULTS . Awareness of AHA guidelines for use of AEDs in children was low in both states in 2003 (29% in Iowa vs 9% in Montana, p< 0.001). After release of the 2005 guidelines, awareness improved significantly in both states but was still significantly greater in Iowa (83% vs. 60 %, p < 0.002). In 2003, less than 20% of respondents in both states reported access to pediatric capable AEDs. Availability of pediatric pads and cables increased significantly in 2006 but remained low in Montana (74% in Iowa vs 37% in Montana, p < 0.001). CONCLUSIONS . At the present time, publication of new or interim guidelines in the scientific literature alone is insufficient to ensure that awareness among providers and that new protocols are implemented. An effective and efficient method to disseminate new pediatric out-of-hospital protocols emergency care to become standard of care in a timely matter should be developed.

Objectives Population-based data for pediatric cardiac arrest are scant and concentrated from large urban areas. The Resuscitation Outcomes Consortium (ROC) Epistry-Cardiac Arrest is a prospective population-based EMS registry of out-of-hospital non-traumatic cardiac arrest (OHCA). The purpose of this study is to examine the characteristics of OHCA in patients Methods Design : Prospective population based cohort study with uniform data definitions. Setting : 11 US and Canadian urban and rural sites participating in the ROC. Population : Persons receive CPR by EMTs and/or receive a bystander AED shock or are pulseless but receive no EMS resuscitation between 12/2005 thru 11/2006. Hypothesis : Survival from pediatric OHCA is less than adult survival. Survival is discharge from hospital. Patients were divided in 3 groups: Results Data from 389 OHCAs in children were submitted: 315 (81%) patients received EMS treatment. Table 1 shows data from all children with OHCA. Table 2 shows EMS-treated OHCAs. Survival of pediatric patients was statistically better than adults for all OHCAs (7.5% vs. 3.9%, p < 0.009) and for EMS-treated OHCAs (9.2% vs 6.9% p = 0.001). EMS scene time was Conclusions Survival from OHCA is better among children than adults, and this current survival rate is better than most previous studies. Bystander CPR is provided for Table 1 Baseline Characteristics for all OHCA Patients Table 2 EMS-Treated OHCA Patients

Introduction The Public Access Defibrillation (PAD) Trial demonstrated improved survival of cardiac arrest victims when automated external defibrillators (AEDs) were combined with CPR by lay responders. These PAD sites incorporated American Heart Association (AHA) recommended elements of a PAD site and the volunteer responders received refresher CPR/AED training. It is unknown if typical community PAD sites maintain these elements after initial program development. The Johnson County Early Defibrillation Task Force (JCEDTF) in Johnson County, IA distributed AEDs throughout the county in 2002. CPR training was provided at program initiation. The purpose of this study was to evaluate the PAD sites 5 years later to assess compliance with AHA recommendations. Methods A 25 point scoring system was developed to assign numerical values to the components of a PAD site: planned and practiced response, links with local EMS, and training of rescuers. Surveys were mailed to all 39 PAD sites. Site visits with tours were conducted at each the site to confirm the survey. Sites were grouped into educational, community, or business sites. Results Thirty two surveys were returned (response rate 82%): 5 educational sites, 13 business and industrial sites and 14 community sites. The Table shows the percentage of points that each group achieved for the components of an AED program. No site had incorporated all the recommended elements: the best sites included only 2/3 of the recommendations. There was a statistical difference in CPR training, with business and industrial sites performing best. Community sites were particularly weak with CPR training. Conclusions PAD sites incorporate approximately half of the elements of an effective PAD program. Business and industrial sites perform slightly better than educational or community sites. CPR training and EMS links are the poorest areas of compliance. These results may indicate that the effectiveness of a PAD site may diminish with time. Table: Percentage of Achieved Points

Summary Objective Electrocardiographic (ECG) rhythm analysis algorithms for cardiac rhythm analysis in automated external defibrillators (AEDs) have been tested against pediatric patient rhythms (patients ≤8 years old) using adult ECG algorithm criteria. However these adult algorithms may fail to detect non-shockable pediatric tachycardias because they do not account for the difference in the rates of normal sinus rhythm and typical tachyarrhythmias in childhood. Methods This study was designed to define shockable and non-shockable rhythm detection criteria specific to pediatric patients to create a pediatric rhythm database of annotated rhythms, to develop a pediatric-based AED rhythm analysis algorithm, and to test the algorithm's accuracy. Pediatric rhythm detection criteria were defined for coarse ventricular fibrillation, rapid ventricular tachycardia, and non-shockable rhythms, including pediatric supraventricular tachycardia. Pediatric rhythms were collected as sustained, classifiable, rhythms ≥9 s in length, and were annotated by pediatric electrophysiologists as clinically shockable or non-shockable based on pediatric criteria. Rhythms were placed into a pediatric rhythm database; each rhythm was converted to digitally accessible, public-domain, MIT rhythm data format. The database was used to evaluate a pediatric-based AED rhythm analysis algorithm. Results Electrocardiographic rhythms from 198 children were recorded. There were 120 shockable rhythms from 49 patients (sensitivity; coarse ventricular fibrillation: 42 rhythms, 100%; rapid ventricular tachycardia: 78 rhythms, 94%), for combined sensitivity of 96.0% (115/120). There were 585 non-shockable rhythms from 155 patients (specificity normal sinus: 208 rhythms, 100%; asystole: 29 rhythms, 100%; supraventricular tachycardia: 161 rhythms, 99%; other arrhythmias: 187 rhythms, 100%), for combined specificity of 99.7% (583/585). Overall accuracy for shockable and non-shockable rhythms was 99.0% (702/709). Conclusions New pediatric rhythm detection criteria were defined and analysis based on these criteria demonstrated both high sensitivity (coarse ventricular fibrillation, rapid ventricular tachycardia) and high specificity (non-shockable rhythms, including supraventricular tachycardia). A pediatric-based AED can detect shockable rhythms correctly, making it safe and exceptionally effective for children.

Background— Early outcomes after tricuspid valve replacement in young children are ill defined. The experience of the Pediatric Cardiac Care Consortium (45 centers, 1984 to 2002) was reviewed to evaluate the results of tricuspid valve replacement in children Methods and Results— Ninety-seven patients who underwent initial tricuspid valve replacement are included in the present analysis. The most frequent cardiac diagnoses were Ebstein’s anomaly (40%), pulmonary atresia (11%), and tetralogy of Fallot (8%). Age at tricuspid valve replacement was 2.9±1.7 years (mean±SD). Mean patient weight was 12.7±6.1 kg. The major outcome was survival to discharge. Associations among age, diagnosis, valve type/size, and outcome were evaluated through the use of χ 2 analysis and logistic regression model fitting approaches. Hospital mortality was 26% and was very high (64%) in patients P 2.5. Other complications included heart block requiring a pacemaker (13%) and thrombosis (5%). Pacemaker implantation was associated with the use of a mechanical valve (23% versus 6% bioprosthetic valve; P =0.01) Conclusions— Tricuspid valve replacement in young children is associated with high mortality, especially in infants

Our patient was diagnosed with familial 2:1 atrioventricularblock and had initially presented with bradycardia and as-sociated shortness of breath and decreased exercise toler-ance at the age of 7. She subsequently underwent an epi-cardial DDD pacemaker implantation with immediateimprovement in symptoms.At age 11, she was noted to have loss of sensing andcapture due to a ventricular lead fracture. Additionally, thegenerator was approaching elective replacement time. Sheunderwent placement of a transvenous DDDR pacemaker.The pocket was created beneath the pectoralis muscle. Us-ing a venogram as a “roadmap”, the axillary vein wasentered at two separate sites through the pocket. Medtronic3830 active fixation leads were placed into the mid septalarea of the right ventricle, and near the right atrial append-age. Additional slack was advanced on both the atrial andventricular lead to allow for growth. Pacing and sensingthresholds were established and adequate values were ob-tained. The proximal end of the lead was secured using thesewing sleeve with 2-0 Prolene (monofilament nonabsorb-able suture) and was then attached to the underlying fascia.The pacemaker generator was placed in the pocket withleads attached. With pacemaker interrogation appropriatepacemaker function was noted without diaphragmatic stim-ulation. The pacemaker was sutured to the underlying fasciawithin the pocket with the 2-0 Prolene, and the pocket wasclosed in three layers.A chest x-ray the following morning showed stable leadplacement with appropriate slack of the lead within theventricle (Figure 1). The pacemaker interrogation prior todischarge demonstrated appropriate pacemaker and leadfunction and a 24-hour ambulatory ECG monitor was es-sentially unremarkable. A sling was worn for the next 3–4days and the patient was instructed not to raise her armabove her shoulder for the next 6 weeks.Five weeks after implantation, the patient noted that herheart rate was low and she experienced twitching of her armthat seemed to coincide with her pacemaker rate. No othersymptoms were reported. She and her mother indicated shehad been compliant with the arm restrictions. She deniedmanipulating the pacemaker area, the device, or the lead.She stated that the incision had itched, but she had been verycareful not to scratch “hard”. Axillary and pocket stimula-tion were noted on physical exam. Device interrogationdemonstrated complete lack of sensing and pacing withnormal lead impedance values. The chest x-ray demonstratedthat the lead had retracted completely out of the heart andappeared to be wrapped around the pacemaker (Figure 2). Thetip of the ventricular lead was at the anterior axillary linewhile the atrial lead remained in place.The ventricular lead was removed and a new lead wasplaced in the manner previously described. During the ven-tricular lead replacement, the sewing sleeve was noted to bein the same position as originally placed, but with the leadpulled completely out of the sleeve. There were no visibleabnormalities of the lead. Since replacement of the lead, nosubsequent lead problems have been found, and the pace-maker system was functioning normally at both her one andthree month follow-up visits.

This publication contains the pediatric and neonatal sections of the 2005 International Consensus on Cardiopulmonary Resuscitation and Emergency Cardiovascular Care Science With Treatment Recommendations (COSTR). The consensus process that produced this document was sponsored by the International Liaison Committee on Resuscitation (ILCOR). ILCOR was formed in 1993 and consists of representatives of resuscitation councils from all over the world. Its mission is to identify and review international science and knowledge relevant to cardiopulmonary resuscitation (CPR) and emergency cardiovascular care (ECC) and to generate consensus on treatment recommendations. ECC includes all responses necessary to treat life-threatening cardiovascular and respiratory events.The COSTR document presents international consensus statements on the science of resuscitation. ILCOR member organizations are each publishing resuscitation guidelines that are consistent with the science in this consensus document, but they also take into consideration geographic, economic, and system differences in practice and the regional availability of medical devices and drugs. The American Heart Association (AHA) pediatric and the American Academy of Pediatrics/AHA neonatal sections of the resuscitation guidelines are reprinted in this issue of Pediatrics (see pages e978–e988).The 2005 evidence evaluation process began shortly after publication of the 2000 International Guidelines for CPR and ECC. The process included topic identification, expert topic review, discussion and debate at 6 international meetings, further review, and debate within ILCOR member organizations and ultimate approval by the member organizations, an Editorial Board, and peer reviewers. The complete COSTR document was published simultaneously in Circulation (International Liaison Committee on Resuscitation. 2005 International Consensus on Cardiopulmonary Resuscitation and Emergency Cardiovascular Care Science With Treatment Recommendations. Circulation. 2005;112(suppl):73–90) and Resuscitation (International Liaison Committee on Resuscitation. 2005 International Consensus Conference on Cardiopulmonary Resuscitation and Emergency Cardiovascular Care Science With Treatment Recommendations. Resuscitation. 2005;67:271–291).Readers are encouraged to review the 2005 COSTR document in its entirety. It can be accessed through the CPR and ECC link at the AHA Web site: www.americanheart.org. The complete publication represents the largest evaluation of resuscitation literature ever published and contains electronic links to more detailed information about the international collaborative process.To organize the evidence evaluation, ILCOR representatives established 6 task forces: basic life support, advanced life support, acute coronary syndromes, pediatric life support, neonatal life support, and an interdisciplinary task force to consider overlapping topics such as educational issues. The AHA established additional task forces on stroke and, in collaboration with the American Red Cross, a task force on first aid. Each task force identified topics requiring evaluation and appointed international experts to review them. A detailed worksheet template was created to help the experts document their literature review, evaluate studies, determine levels of evidence, develop treatment recommendations, and disclose conflicts of interest. Two evidence evaluation experts reviewed all worksheets and assisted the worksheet reviewers to ensure that the worksheets met a consistently high standard.A total of 281 experts completed 403 worksheets on 275 topics, reviewing more than 22000 published studies. In December 2004 the evidence review and summary portions of the evidence evaluation worksheets, with worksheet author conflict of interest statements, were posted on the Internet at www.C2005.org, where readers can continue to access them. Journal advertisements and e-mails invited public comment.Two hundred forty-nine worksheet authors (141 from the United States and 108 from 17 other countries) and additional invited experts and reviewers attended the 2005 International Consensus Conference for presentation, discussion, and debate of the evidence. All 380 participants at the conference received electronic copies of the worksheets. Internet access was available to all conference participants during the conference to facilitate real-time verification of the literature. Expert reviewers presented topics in plenary, concurrent, and poster conference sessions with strict adherence to a novel and rigorous conflict of interest process. Presenters and participants then debated the evidence, conclusions, and draft summary statements. Wording of science statements and treatment recommendations was refined after further review by ILCOR member organizations and the international editorial board. This format ensured that the final document represented a truly international consensus process.The COSTR manuscript was ultimately approved by all ILCOR member organizations and by an international editorial board. The AHA Science Advisory and Coordinating Committee and the editor of Circulation obtained peer reviews of this document before it was accepted for publication.The most important changes in recommendations for pediatric resuscitation since the last ILCOR review in 2000 include: Increased emphasis on performing high quality CPR: “Push hard, push fast, minimize interruptions of chest compression; allow full chest recoil, and don't provide excessive ventilation” Recommended chest compression-ventilation ratio: For lone rescuers with victims of all ages: 30:2For health care providers performing 2-rescuer CPR for infants and children: 15:2 (except 3:1 for neonates)Either a 2- or 1-hand technique is acceptable for chest compressions in childrenUse of 1 shock followed by immediate CPR is recommended for each defibrillation attempt, instead of 3 stacked shocksBiphasic shocks with an automated external defibrillator (AED) are acceptable for children 1 year of age. Attenuated shocks using child cables or activation of a key or switch are recommended in children Some of the most important changes in recommendations for neonatal resuscitation since the last ILCOR review in 2000 include less emphasis on using 100% oxygen when initiating resuscitation, de-emphasis of the need for routine intrapartum oropharyngeal and nasopharyngeal suctioning for infants born to mothers with meconium staining of amniotic fluid, proven value of occlusive wrapping of very low birth weight infants The scientific evidence supporting these recommendations is summarized in the neonatal document (see pages e978–e988).

Objective: This post-market, observational study is intended to evaluate reported uses of pediatric pads that reduce the energy delivered by some adult automated external defibrillators (AEDs) so that they may be used with pediatric patients. Methods: Users of the pediatric pads were asked to report any use of the pads, even if no shock was delivered and to provide detailed information about the event, caregiver and the patient. Results: Reports of the use of pediatric pads have been received and confirmed for 27 patients, age range 0 days to 23 years, median 2 years. Ventricular fibrillation (VF) was reported in eight cases, age range 4.5 months to 10 years, median 3 years. Shocks were delivered to all VF patients, the average shock number was 1.9, range 1–4. All patients had termination of VF, were admitted to the hospital and five survived to hospital discharge. Non-shockable rhythms were reported in 16 patients, and the AED appropriately did not advise a shock. Eleven of these patients had asystole or PEA as their initial rhythm and did not survive to hospital discharge. One report contained no additional information other than that the patient did not survive, and in two other reports, the pads were not applied to patients. Conclusions: Voluntary reports of the use of attenuated pediatric defibrillation pads indicate the devices performed appropriately. All eight VF patients had termination of VF and five survived to hospital discharge. These data support the rapid deployment of AEDs for young children as well as adolescents and adults. Since the pediatric pads are available and deliver an appropriate dose for children, their use should be strongly encouraged.

Background— Prosthesis survival, growth, and functional status after initial mechanical mitral valve replacement (MVR) in children Methods and Results— The experience of the Pediatric Cardiac Care Consortium (45 centers, 1982 to 1999), which included 102 survivors after initial MVR, was analyzed. Median follow-up: 6.0 years (interquartile range: 3.0 to 10.6 years; 96% complete). Twenty-nine survivors had undergone a second MVR at an interval of 4.8±3.8 years after initial MVR. Reasons for second MVR were prosthetic valve stenosis 24 (83%), thrombosis 4 (14%), and endocarditis 1 (3%). For those who had second MVR, prosthesis sizes were: first MVR 19±2 mm and second MVR 22±3 mm, and their body weight increased from 7.4±2.8 kg to 16.8±10.5 kg. To identify risk factors for having a second MVR, the 29 second MVR survivors were compared with the 73 who did not have a second MVR on first-MVR demographic and perioperative variables. By univariate analysis, patients with shorter prosthesis survival were younger, weighed less, had smaller prostheses, greater ratio of prosthesis size:body weight, were less likely to have a St. Jude prosthesis and more likely to have Shone’s syndrome. By multivariate analysis prosthesis survival was predicted only by first MVR age: odds ratio (OR) 7.7 (95% confidence interval [CI] 2.6–22.7) and prosthesis size: OR 6.8 (95% CI 2.6–18.2). High risk patients (age P =0.017). An estimate of current New York Heart Association (NYHA) functional status was class 1 in 76%, class 2 in 22%, and classes 3 or 4 in 2%. Conclusion— Prosthesis survival can be predicted based on first MVR age and prosthesis size. Somatic growth is comparable regardless of the need for second MVR. There is an increment in prosthesis size at second MVR, suggesting continued annular growth. Significant limitation of function after MVR is uncommon. MVR may be an appropriate strategy for children

Atherosclerotic cardiovascular disease remains the leading cause of both death and disability in North America. Evidence that most cardiovascular disease is preventable led to development of the American Heart Association’s initial “Guide to the Primary Prevention of Cardiovascular Disease” in 1996 and the updated version in 2002. Those guidelines do not address prevention in children, a group for whom primary prevention should hold the most promise. Emergence of multiple lines of evidence with regard to the importance of known risk factors for atherosclerotic disease in children and young adults has provided the impetus to develop guidelines for primary prevention in this young population. Pathological studies have shown that both the presence and extent of atherosclerotic lesions at autopsy after unexpected death of children and young adults correlate positively and significantly with established risk factors, namely low-density lipoprotein cholesterol, triglycerides, systolic and diastolic blood pressure, body mass index, and presence of cigarette smoking. Findings from the Bogalusa study indicate that as the number of cardiovascular risk factors increases, so does the pathological evidence for atherosclerosis in the aorta and coronary arteries beginning in early childhood. Electron beam computed tomography of coronary artery calcium and increased carotid artery intima-media thickness, an ultrasound measure of carotid artery atherosclerosis, have been evaluated in 29- to 39-year-olds monitored from 4 years of age. Significant risk predictors for coronary artery calcium were obesity and elevated blood pressure in childhood and increased body mass index and dyslipidemia as young adults. Multiple epidemiological studies have demonstrated a disturbing increase in the prevalence of obesity beginning in childhood, with at least 22% of 6- to 17-year-olds diagnosed as overweight. This is a cause for particular concern because of the strong association between obesity and hypertension, dyslipidemia, and type II diabetes mellitus beginning in childhood. Long-term follow-up studies have demonstrated …

Background Short- and long-term outcomes after prosthetic mitral valve replacement (MVR) in children aged Methods and Results MVR was performed 176 times on 139 patients. Median follow-up was 6.2 years (range 0 to 20 years, 96% complete). Age at initial MVR was 1.9±1.4 years. Complications after initial MVR included heart block requiring pacemaker (16%), endocarditis (6%), thrombosis (3%), and stroke (2%). Patient survival was as follows: 1 year, 79%; 5 years, 75%; and 10 years, 74%. The majority of deaths occurred early after initial MVR, with little late attrition despite repeat MVR and chronic anticoagulation. Among survivors, the 5-year freedom from reoperation was 81%. Age-adjusted multivariable predictors of death include the presence of complete atrioventricular canal (hazard ratio 4.76, 95% CI 1.59 to 14.30), Shone’s syndrome (hazard ratio 3.68, 95% CI 1.14 to 11.89), and increased ratio of prosthetic valve size to patient weight (relative risk 1.77 per mm/kg increment, 95% CI 1.06 to 2.97). Age- and diagnosis-adjusted prosthetic size/weight ratios predicted a 1-year survival of 91% for size/weight ratio 2, 79% for size/weight ratio 3, 61% for size/weight ratio 4, and 37% for size/weight ratio 5. Conclusions Early mortality after MVR can be predicted on the basis of diagnosis and the size/weight ratio. Late mortality is low. These data can assist in choosing between MVR and alternative palliative strategies.

Background: The Long QT Syndrome (LQTS) is a genetic channelopathy with life-threatening implications. The LQT3 form of this disease is caused by mutations of the SCN5A sodium-channel gene. A specific mutation, SCN5A:ΔKPQ, is associated with repetitive reopenings of the sodium channel and prolonged inward current. This dominant inward current is manifest on the electrocardiogram as QT prolongation. Flecainide is a potent blocker of the open sodium channel. Methods and Results: The effect of flecainide on the duration of the QT-interval and the T-wave morphology was systematically evaluated in five male patients age 2–64 years having the SCN5A:ΔKPQ mutation. After baseline electrocardiograms were obtained, low-dose oral flecainide was administered for 48 hours. Serial electrocardiograms and blood flecainide levels were obtained during flecainide therapy. The QTc interval decreased on average by 104 ms, from a baseline value of 565 ± 60 ms to 461 ± 23 ms (P < 0.04) at a mean flecainide level of 0.28 ± 0.08 mg/L, with shortening of the QTonset interval (P < 0.003) and normalization of T-wave morphology. The effects of flecainide were compared with oral mexiletine in two patients, with flecainide showing greater QTc shortening and more complete normalization of repolarization. No adverse side effects or proarrhythmia were observed with flecainide in this study. Conclusion: Low-dose, oral flecainide consistently shortened the QTc interval and normalized the repolarization T-wave pattern in five LQT3 patients with SCN5A:ΔKPQ mutation. This preliminary study indicates that low-dose flecainide is a promising therapeutic agent for LQTS patients with the SCN5A:ΔKPQ sodium channel mutation. A.N.E. 2001;6(2):153–158

Although medications can potentially cause sudden, unexpected death by a variety of mechanisms, cardiac arrhythmias are the most frequent cause. Reports of sudden deaths of children and adolescents treated with psychotropic medications have raised concerns regarding the appropriateness of this therapy, as well as the advisability of baseline and periodic electrocardiographic monitoring of such patients. Stimulants such as the amphetamines and methylphenidate cause slight but clinically insignificant increases in heart rate and blood pressure. The tricyclic antidepressants imipramine and desipramine have been associated with at least seven reported deaths in young patients. The selective serotonin reuptake inhibitors have minimal cardiovascular effects; deaths have been rare, even with massive overdose.

Objective: The purpose of this investigation was to establish a model system to facilitate identification of the sympathetic neuronal factor(s) that promotes improved contractility in neonatal cardiac myocytes. Conditioned medium from PC12 cells with sympathetic phenotype served as the source of the neuronal factor. Methods: Contraction frequency, amplitude and velocity of cultured neonatal rat cardiac myocytes were measured by online video analysis. Interventions included in vitro sympathetic innervation, exposure to PC12 conditioned medium, neurotransmitters and antagonists. Metabolic activity was assayed by 2-deoxyglucose uptake. Troponin T isoform expression was analyzed by SDS–polyacrylamide gel electrophoresis. Results: Medium conditioned by neuronal PC12 cells induced contractility changes similar to those induced by in vitro sympathetic innervation. These effects of PC12 conditioned medium and innervation were not suppressed by adrenergic or muscarinic antagonists nor reproduced by neuropeptide Y or somatostatin. Neuronal PC12 conditioned medium but not chromaffin PC12 conditioned medium, increased metabolic activity of the myocytes as detected by [3H]-2-deoxyglucose, indicating that the effect was specific to the neuronal PC12 cells. The in vitro switch of troponin T isoform expression was not altered by exposure to PC12 conditioned medium. Conclusions: Increased contractile function induced by sympathetic innervation is reproduced by PC12 conditioned medium, but neither is mediated by sympathetic or muscarinic neurotransmitters. Troponin T isoform expression is not related to the contractility changes. This model system will allow identification of the factor(s).

Reports of sudden deaths of children and adolescents treated with psychotropic medications have raised concerns regarding the appropriateness of this therapy, as well as the advisability of baseline and periodic electrocardiographic (ECG) monitoring of such patients.1 2 3 4 What follows is a review of the drug effects on the ECG, cardiovascular effects of the commonly used psychotropic medications in children and adolescents, a summary of potentially dangerous drug interactions, and recommendations for cardiovascular monitoring. Although medications can potentially cause sudden, unexpected death by a variety of mechanisms (eg, seizures, central nervous system depression, or coronary artery spasm), cardiac arrhythmias are the most frequent cause. In particular, a unique form of ventricular tachycardia termed torsade de pointes has been recognized as the arrhythmia responsible for the so-called proarrhythmic effect of several antiarrhythmia drugs, and recent evidence has pointed to a similar mechanism in syncope and deaths related to other medications5 and in the familial long-QT syndromes.6 The common feature of these conditions is delayed repolarization of the myocardium (related to abnormal sodium or potassium currents) with resultant prolongation of the QT interval of the ECG. This appears to leave the myocardium vulnerable to ventricular tachycardia, primarily in the setting of bradycardia but occasionally in association with exercise. Other ECG abnormalities, such as sinus node depression, …

In 1996 the American Heart Association published a scientific statement on screening for cardiovascular abnormalities in young athletes participating in organized sports programs in US high schools and colleges. “Cardiovascular Preparticipation Screening of Competitive Athletes” ( Circulation. 1996;94:850–856) served as a critique of current and past screening practices and offered several specific recommendations for future screening endeavors, including the content of history and physical examination questionnaires, the nature and qualifications of designated examiners, and the timing of preparticipation examinations. This statement has become a well-known guideline endorsed by other medical and exercise science organizations. However, the recommendation for timing of preparticipation evaluations has become the source of some discussion. In the original statement, the writing group recommended that …

Operative mortality for 262 infants with interruption of the aortic arch repaired from 1982 to June 1993 has remained constant at about 35%. Major coexistent cardiac malformations, type B interruption, or staged repair are risk factors for mortality.

We conclude that to minimize TTI, preapplied self-adhesive electrode pads should be at least 5.8 cm in diameter when used for pediatric patients. The 5.8 cm pad was easily applied to infants. Older children could easily accommodate the 7 cm pad. Therefore, we recommend a pad diameter of 5.8 cm for infants

Sympathethic innervation of the heart develops during late gestation and is not fully mature until the end of the neonatal period. The onset of neuroeffector transmission coincides with the cessation of myocyte mitosis (Claycomb, 1976) and the beginning of cellular hypertrophy (Clubb and Bishop, 1984). This process must occur during normal development and growth but also occurs pathologically in the setting of heart disease and hypertension. Although hemodynamic load is a major stimulus for cardiac hypertrophy, it is not the sole regulatory mechanism as evidenced by hypertrophic cardiomyopathy, which occurs in the presence of presumed normal hemodynamics.

Sympathetic innervation is known to increase heart size in the immature animal, yet the mechanism for this growth remains to be established. This comparative study stereologically quantified the volume of cultured neonatal ventricular myocytes with and without in vitro sympathetic innervation to isolate the mechanisms regulating cardiac growth. Since ventricular myocyte size at birth differs between the spontaneously hypertensive rat (SHR) and the normotensive Wistar-Kyoto (WKY), we questioned whether SHR myocytes respond differently than WKY myocytes to innervation. Four groups of ventricular myocytes from each strain were compared: myocytes grown alone, myocytes innervated by cultured sympathetic neurons, innervated myocytes exposed to adrenoceptor blockade, and non-innervated myocytes in co-culture dishes. Volumes for the myocyte, nucleus, cytoplasm, mitochondria, sarcomeres and other cellular organelles were assessed within each population and between populations. Relative volumes were determined for the mitochondria, sarcomeres, and other cellular components within the cytoplasm. Innervated WKY myocytes were 38% larger than control myocytes (P < 0.0004). This growth was not blocked by adrenoceptor blockade (P = 0.89 vs. innervated) and was present in the non-innervated myocytes distant from the neurons in the co-cultures (P = 0.39 vs. innervated). SHR myocytes were 36% larger than WKY myocytes (P < 0.009) but did not increase with innervation (P = 0.48). SHR myocyte size was also unaffected by adrenoceptor blockade (P = 0.39) or presence of the neurons in the culture dish (P = 0.53). Neonatal WKY ventricular myocyte growth can be provoked in vitro by sympathetic innervation via regulatory mechanisms independent of neuroeffector transmission or anatomic contact, whereas volume of neonatal SHR myocytes is unaltered by sympathetic coculture. These findings are significant for understanding normal as well as aberrant cardiomyocyte growth.

The overall incidence of ventricular tachycardia/ventricular fibrillation (VT/VF) as the first recorded electrical rhythm in out-of-hospital cardiac arrest has declined from ~70% to ~25% over the past 30 years. This change has been attributed to primary and secondary prevention of cardiovascular disease and VT/VF. We evaluated whether the incidence of VT/VF as first recorded rhythm differed by location among bystander-applied automatic external defibrillator (AED) patients and emergency medical services (EMS)-witnessed cardiac arrests.

A three-dimensional analysis to evaluate structural changes in cultured cardiac myocytes following adrenergic innervation was performed using stereological techniques formerly limited to cells in tissue and organs. Cell volumes were calculated for two groups of cells at 96 hours in culture: isolated myocytes and myocytes innervated with adrenergic neurons. Relative and absolute volumes of the nucleus, cytoplasm, and cell were quantified by systematically sampling sections throughout the cell and by point count sampling techniques. Volumetric estimates were similarly determined for the mitochondria, sarcomeres, and other cellular components in the cytoplasm. Data were analyzed with ANOVA and randomized block design to control for variation among the cultures. Adrenergic innervation produced a 44% increase in cell volume, X±SEM, (3,344±196 μm3 to 4,816±400 μm3, P = 0.007). The absolute volume of mitochondria significantly increased after innervation (521±42 μm3 to 744 ± 54 μm3, P

Four children aged 2 to 15 years are described who had Stokes-Adams syndrome as the primary cardiac manifestation of complete heart block. All had mild, nonspecific antecedent symptoms without signs of congestive heart failure. Viral neutralizing antibody titers (coxsackievirus B1 and B2) rose significantly in two patients. Electrocardiography demonstrated complete heart block associated with one or more of the following: right bundle branch block, left anterior hemiblock, left bundle branch block, intraventricular conduction delay, and ventricular standstill. In addition, second-degree heart block was seen during the initial or resolving phases of illness. To prevent further life-threatening episodes of Stokes-Adams syndrome, temporary transvenous pacemakers were placed. Two patients with persisting complete heart block required permanent pacemakers. At last examination, the remaining two patients had sinus rhythm and normal conduction. Nonsurgical acquired complete heart block is unusual in childhood, and may be the only manifestation of myocarditis. Extreme bradycardia, which causes syncope, requires immediate temporary cardiac pacing; if the bradycardia persists, a permanent pacemaker should be implanted.

A patient with sick sinus syndrome following the Senning operation was also found to have concealed atrial parasystole during intracardiac electrophysiological study. This atrial parasystolic pattern could be converted to atrial bigeminy by changing the atrial drive rate.

Single-chamber, rate-responsive pacing enables pacemaker-dependent patients to increase their heart rates with activity, thus mimicking the physiologic chronotropic response to exercise. Studies have shown that rate-responsive pacing in adults significantly improves both exercise capacity during stress testing and symptoms during regular daily activity.1 The experience with rate-responsive pacing in the pediatric population is limited. Previous studies have demonstrated increased exercise capacity and subjective improvement of symptoms with rate-responsive pacing.2,3 We report our experience with 5 pediatric patients with ventricular rate-responsive pacing who were studied in both the fixed-rate and rateresponsive modes.

We examined 51 cardiac specimens to test the hypothesis that the size of the foramen ovale is directly proportional to the relative volume of transatrial blood flow during cardiac morphogenesis. Included in the study were 18 normal specimens, five with tricuspid atresia, four with pulmonary atresia and intact ventricular septum, nine with secundum atrial septal defect, eight with simple coarctation of the aorta and seven with aortic stenosis. The areas of the foramen ovale and atrial septum were measured and the ratio of foramen ovale to atrial septum was calculated. The area of the atrial septum did not differ significantly among the six groups. Normal specimens had a ratio of 0.19 +/- 0.07. Specimens with tricuspid atresia and pulmonary atresia had ratios of 0.43 +/- 0.01 and 0.38 +/- 0.10, respectively (p less than 0.05). The ratio in specimens with coarctation of the aorta was 0.15 +/- 0.07 (NS). Specimens with aortic stenosis had a ratio of 0.11 +/- 0.06 (p less than 0.05). Specimens with secundum atrial septal defects had a ratio of 0.37 +/- 0.08 (p less than 0.05). These data indicate that the foramen ovale is a marker of transatrial blood flow in utero. Secundum atrial septal defects may be a consequence of increased transatrial blood flow.

The indirectly estimated sinoatrial conduction time has been widely used in children to assess sinus node (SN) function, but has never been validated in pediatric patients. Using a standard quadripolar catheter with high amplification and low-pass filters, SN electrograms were recorded in 11 of 26 (42%) pediatric cardiac patients (age range 10 months to 18 years) with normal SN function. The sinoatrial conduction time was measured directly from the SN electrogram and estimated indirectly by the premature stimulation and continuous pacing methods. The direct sinoatrial conduction time (15 to 91 ms) in these 11 patients (ages 1 to 18 years) correlated well with that estimated by the premature stimulation method (r = 0.81, n = 9, p less than 0.01). There was a poor correlation between the direct and continuous pacing methods (r = 0.53, n = 8, p = 0.17). It was concluded that SN electrograms can be recorded in children and that there is a good correlation between the directly measured sinoatrial conduction time and the indirectly estimated sinoatrial conduction time by the premature stimulation method.