Objectives To assess the performance of 3 risk scores from Japan that were developed to predict, in children with Kawasaki disease, resistance to intravenous immunoglobulin (IVIG) treatment. Study ...design We used data from a randomized trial of pulsed steroids for primary treatment of Kawasaki disease to assess operating characteristics of the 3 risk scores, and we examined whether steroid therapy lowers the risk of coronary artery abnormalities in patients prospectively classified as IVIG resistant. Results For comparability with published cohorts, we analyzed the data of 99 patients who were not treated with steroids (16% IVIG-retreated) and identified male sex, lower albumin level, and higher aspartate aminotransferase level as independent risk factors for IVIG resistance. The Kobayashi score was similar in IVIG-resistant and -responsive patients, yielding a sensitivity of 33% and specificity of 87%. There was no interaction of high-risk versus low-risk status by treatment received (steroid versus placebo) with any of the 3 risk score algorithms. Conclusion Risk-scoring systems from Japan have good specificity but low sensitivity for predicting IVIG resistance in a North American cohort. Primary steroid therapy did not improve coronary outcomes in patients prospectively classified as being at high-risk for IVIG resistance.
Objective To describe common associated symptoms within the 10 days before diagnosis in subjects enrolled in the Pediatric Heart Network's trial of steroid therapy in Kawasaki disease (KD). Study ...design Patients with acute KD were enrolled between days 4 and 10 of illness at 8 centers between 2002 and 2004. We defined common associated symptoms as those occurring in ≥10% of patients. Principal clinical criteria for KD were not included in this analysis. Results Among 198 patients, irritability was reported in 98 (50%), vomiting in 88 (44%), decreased food/fluid intake in 73 (37%), cough in 55 (28%), diarrhea in 52 (26%), rhinorrhea in 37 (19%), weakness in 37 (19%), abdominal pain in 35 (18%), and joint pain (arthralgia or arthritis) in 29 (15%). One or more gastrointestinal symptom (vomiting, diarrhea, or abdominal pain) was present in 120 patients (61%) and 69 patients (35%) had ≥ 1 respiratory symptom (rhinorrhea or cough). Conclusions Nonspecific symptoms occur commonly in children with KD. To reduce delays in diagnosis, clinicians should be educated that such symptoms may comprise a significant component in the chief complaint.
Development of pediatric medications and devices is complicated by differences in pediatric physiology and pathophysiology (both compared with adults and within the pediatric age range), small ...patient populations, and practical and ethical challenges to designing clinical trials. This article summarizes the discussions that occurred at a Cardiac Safety Research Consortium–sponsored Think Tank convened on December 10, 2010, where members from academia, industry, and regulatory agencies discussed important issues regarding pediatric cardiovascular safety of medications and cardiovascular devices. Pediatric drug and device development may use adult data but often requires additional preclinical and clinical testing to characterize effects on cardiac function and development. Challenges in preclinical trials include identifying appropriate animal models, clinically relevant efficacy end points, and methods to monitor cardiovascular safety. Pediatric clinical trials have different ethical concerns from adult trials, including consideration of the subjects' families. Clinical trial design in pediatrics should assess risks and benefits as well as incorporate input from families. Postmarketing surveillance, mandated by federal law, plays an important role in both drug and device safety assessment and becomes crucial in the pediatric population because of the limitations of premarketing pediatric studies. Solutions for this wide array of issues will require collaboration between academia, industry, and government as well as creativity in pediatric study design. Formation of various epidemiologic tools including registries to describe outcomes of pediatric cardiac disease and its treatment as well as cardiac effects of noncardiovascular medications, should inform preclinical and clinical development and improve benefit-risk assessments for the patients. The discussions in this article summarize areas of emerging consensus and other areas in which consensus remains elusive and provide suggestions for additional research to further our knowledge and understanding of this topic.
Protecting the Heart of the American Athlete Lawless, Christine E., MD, FACC, FACSM; Asplund, Chad, MD, FACSM; Asif, Irfan M., MD ...
Journal of the American College of Cardiology,
11/2014, Letnik:
64, Številka:
20
Journal Article
Recenzirano
Odprti dostop
The American College of Cardiology (ACC) Sports and Exercise Cardiology Section convened the "Think Tank to Protect the Heart of the American Athlete and Exercising Individual" on October 18, 2012, ...in Washington, DC.\n Asif University of Tennessee Family and Sports Medicine--Assistant Professor; Fellowship Director, Sports Medicine None None None None None None Ron Courson University of Georgia--Senior Associate Athletic Director None None None None None None Michael S. Emery Carolina Cardiology Consultants None None None None None None Anthon R, Fuisz Medstar Washington Hospital Center-Director, Cardiac MRI None None None None None None Richard J. Kovacs Krannert Institute of Cardiology-Professor of Clinical Medicine Biomedical Systems Insight Pharmaceuticals Theravancedagger Xenoport None None Biotie (DSMB) Eli Lilly (DSMB)dagger Cook Incorporated Med Institutedagger None Silvana M. Lawrence Baylor College of Medicine--Associate Professor, Department of Pediatrics, Section of Cardiology None None None None None None Benjamin D. Levine Institute for Exercise and Environmental Medicine None None None None None Defendant, 2013, Postural Orthostatic Tachycardia Syndrome Mark S. Link Tufts Medical Center None None None None None None Matthew W. Martinez Lehigh Valley Health Network None None None None None None G. Paul Matherne University of Virginia Health Sciences Center-Division Chief of Pediatric Cardiology None None None None None None Brian Olshansky University of Iowa Hospitals-Professor of Medicine Arrhythmia Grand Roundslow * BioControl Boehringer Ingleheim Boston Scientific (guidant) Combined Medicare Medicaid Services Daiichi Sankyo Gerson Lehman Medtronicdagger Sanofi Aventis None None Amarin (DSMB) Boston Scientific (DSMB) Sanofi Aventis (DSMB) Boston Scientific Executive Health Resourcesdagger Thompson Reuterslow * Defendant, 2013 Event Monitors Third Party, 2012, Cardiac Arrest William O. Roberts University of Minnesota Medical School-Professor, Department of Family Medicine and Community Health None None None None None None Lisa Salberg Hypertrophic Cardiomyopathy Association-Chief Executive Officer None None None None None None Victoria L. Vetter Children's Hospital of Philadelphia Division of Cardiology-Professor of Pediatrics None None None None None None Robert A. Vogel University of Colorado-Professor of Medicine Pritikin Longevity Center National Football Leaguedagger None None Sanofidagger None None Jim Whitehead American College of Sports Medicine-Executive Vice President/Chief Executive Officer * This table represents all relationships of committee members with industry and other entities that were reported by authors, including those not deemed to be relevant to this document, at the time this document was under development.
Children with long QT syndrome (LQTS) live with the risk of sudden death, activity restrictions, and the need for daily medications. We sought to evaluate the quality of life (QOL), self-perception, ...and behavior of patients with LQTS as perceived by both patients and their parents and identify predictors of lower QOL. QOL (Pediatric QOL Inventory PedsQL and Pediatric Cardiac Quality of Life Inventory PCQLI), self-perception, and behavioral inventories were completed by patients with LQTS and their parents. Comparison of PedsQL scores was made to published data for healthy children using t tests, and PCQLI scores were compared with those of patients with differing complexity of congenital heart disease. Mixed modeling was used for multivariable analysis. Sixty-one patients with LQTS were evaluated (age 13.6 ± 3.0 years; male 49%). Compared with healthy children, the PedsQL Total, Psychosocial, and Physical Health Summary scores were significantly lower for patients with LQTS and parent proxy reports (p ≤0.001). In general, PCQLI scores of patients with LQTS and parents were similar to those of patients with tetralogy of Fallot (p ≥0.2), lower than those of patients with bicuspid aortic valve (p ≤0.02), and higher than those of patients with single ventricle (p ≤0.03). Lower patient and parent PCQLI scores were associated with internalizing problems. For parents, the presence of a cardiac device and medication side effects were additionally associated with lower PCQLI scores. In conclusion, patients with LQTS and their parents report lower QOL than normal children secondary to physical and psychosocial factors. Increasing focus on the psychological well-being of these patients is needed in an effort to improve their QOL.
Objectives Our objective was to determine the relationship between functional outcome and abnormalities of heart rate and rhythm after the Fontan operation. Methods The National Heart, Lung, and ...Blood Institute Pediatric Heart Network conducted a cross-sectional analysis of patients who had undergone a Fontan procedure at the 7 network centers. Analysis was based on 521 patients with an electrocardiogram (n = 509) and/or bicycle exercise test (n = 404). The Child Health Questionnaire parent report and the oxygen consumption at the anaerobic threshold were used as markers of functional outcome. Results Various Fontan procedures had been performed: intracardiac lateral tunnel (59%), atriopulmonary connection (14%), extracardiac later tunnel (13%), and extracardiac conduit (11%). Prior volume unloading surgery was performed in 389 patients: bidirectional Glenn (70%) and hemi-Fontan (26%). A history of atrial tachycardia was noted in 9.6% of patients and 13.1% of patients had a pacemaker. Lower resting heart rate and higher peak heart rate were each weakly associated with better functional status, as defined by higher anaerobic threshold ( R = −0.18, P = .004, and R = 0.16, P = .007, respectively) and higher Child Health scores for physical functioning ( R = −0.18, P < .001, and R = 0.17, P = .002, respectively). Higher anaerobic threshold was also independently associated with younger age and an abnormal P-axis. Resting bradycardia was not associated with anaerobic threshold or Child Health scores. Conclusions In pediatric patients (6–18 years) after the Fontan procedure, a lower resting heart rate and a higher peak heart rate are each independently associated with better physical function as measured by anaerobic threshold and Child Health scores. However, these correlations are weak, suggesting that other, nonrhythm and nonrate, factors may have a greater impact on the functional outcome of pediatric patients after the Fontan operation.
Background Cardiovascular conditions rank sixth in causes of death in 1- to 19-year-olds. Our study is the first analysis of the cardiovascular death data set from the National Center for the Review ...and Prevention of Child Deaths, which provides the only systematic collection of cardiovascular deaths in children. Methods We developed an analytical data set from the National Center for the Review and Prevention of Child Deaths database for cardiovascular deaths in children 0 to 21 years old, reviewing 1,098 cases from 2005 to 2009 in 16 states who agreed to participate. Results Cardiovascular cases were aged 4.8 ± 6.6 years; 55.3%, ≤1 year; 24.6%, ≥10 years; male, 58%; white, 70.5%; black, 22.3%; Hispanic, 19.5%. Prior conditions were present in 48.5%: congenital heart disease, 23%; cardiomyopathies, 4.6%; arrhythmia, 1.7%; and congestive heart failure, 1.6%. Deaths occurred most frequently in urban settings, 49.2%; and in the hospital, 40.4%; home, 26.1%; or at school/work/sports, 4.8%. Emergency medical services were not evenly distributed with differences by age, race, ethnicity, and area. Autopsies (40.4%) occurred more often in those >10 years old (odds ratio OR 2.9), blacks (OR 1.6), or in those who died at school/work/sports (OR 3.9). The most common cardiovascular causes of death included congenital heart disease, 40.8%; arrhythmias, 27.1%; cardiomyopathy, 11.8%; myocarditis, 4.6%; congestive heart failure, 3.6%; and coronary artery anomalies, 2.2%. Conclusions Our study identified differences in causes and frequencies of cardiovascular deaths by age, race, and ethnicity. Prevention of death may be impacted by knowledge of prior conditions, emergency plans, automated external defibrillator programs, bystander cardiopulmonary resuscitation education, and by a registry for all cardiovascular deaths in children.
Background Wyman W. Lai, MD, MPH, and Victoria L. Vetter, MD, MPH. The Pediatric Heart Network (PHN), funded under the U.S. National Institutes of Health–National Heart, Lung, and Blood Institute ...(NIH–NHLBI), includes two Clinical Research Skills Development (CRSD) Cores, which were awarded to The Children's Hospital of Philadelphia and to the Morgan Stanley Children's Hospital of New York–Presbyterian. To provide information on how to develop a clinical research career to a larger number of potential young investigators in pediatric cardiology, the directors of these two CRSD Cores jointly organized a one-day seminar for fellows and junior faculty from all of the PHN Core sites. The participants included faculty members from the PHN and the NHLBI. The day-long seminar was held on April 29, 2009, at the NHLBI site, immediately preceding the PHN Steering Committee meeting in Bethesda, MD. Methods The goals of the seminar were 1) to provide fellows and early investigators with basic skills in clinical research 2) to provide a forum for discussion of important research career choices 3) to introduce attendees to each other and to established clinical researchers in pediatric cardiology, and 4) to publish a commentary on the future of clinical research in pediatric cardiology. Results The following chapters are compilations of the talks given at the 2009 PHN Clinical Research Skills Development Seminar, published to share the information provided with a broader audience of those interested in learning how to develop a clinical research career in pediatric cardiology. The discussions of types of clinical research, research skills, career development strategies, funding, and career management are applicable to research careers in other areas of clinical medicine as well. Conclusions The aim of this compilation is to stimulate those who might be interested in the research career options available to investigators.
Objective Risk factors for poor outcome with congenital complete heart block include prematurity, low birth weight, hydrops, low ventricular rates, and congenital heart disease. In this group, ...medical therapy is often ineffective, pacing is technically challenging, and mortality exceeds 80%. The purpose of this study is to assess outcomes of patients with congenital complete heart block who were paced in the first 24 hours after birth owing to the presence of known risk factors. Methods We performed a retrospective review of patients with congenital complete heart block paced in the first 24 hours after birth at our institution between November 1, 1995, and July 31, 2007. Results Thirteen patients were identified, 4 of whom had heterotaxy syndrome. Eleven patients had temporary epicardial pacing wires placed; 2 received permanent pacemakers as the initial mode of pacing. There were 7 deaths (54% mortality) at a mean age of 19.9 ± 19 days. Among 7 patients with structural heart disease, there was 1 survivor. Among 6 patients with structurally normal hearts, there were 5 survivors ( P = .025). Patients with temporary wires who survived to permanent pacemaker implantation (6/11) used their temporary leads for 33.8 ± 18.3 days. Conclusions In the severely affected fetus with congenital complete heart block and significant structural heart disease, outcomes remain poor; however, neonates with congenital complete heart block and structurally normal hearts who are monitored antenatally and delivered in a planned fashion at an institution capable of early pacing can have favorable outcomes. The use of temporary pacing wires is an option in the management of these patients.
Background In children, sudden cardiac arrest (SCA) is associated with structural and electrical cardiac abnormalities. No studies have systematically screened healthy school children in the United ...States for conditions leading to SCA to identify those at risk. Methods From June 2006 to June 2007, we screened 400 healthy 5- to 19-year-olds (11.8 ± 3.9 years) in clinical offices at The Children's Hospital of Philadelphia using a medical and family history questionnaire, weight, height, blood pressure, heart rate, cardiac examination, electrocardiogram (ECG), and echocardiogram (ECHO). Our goals were to determine the feasibility of adding an ECG to history and physical examination and to identify a methodology to be used in a larger multicenter study. A secondary objective was to compare identification of cardiovascular abnormalities by history and physical examination, ECG, and ECHO. Results Previously undiagnosed cardiac abnormalities were found in 23 subjects (5.8%); an additional 20 (5%) had hypertension. Potentially serious cardiac conditions were identified in 10 subjects (2.5%); 7 were suspected or identified by ECG and 3 more only by ECHO. Only 1 of the 10 had symptoms (previously dismissed); none had a positive family history. Conclusions It is feasible to screen for conditions associated with SCA in healthy children by adding ECG to history and physical examination. In this nongeneralizable sample, ECG identified more cases compared to history and physical examination alone, with further augmentation from ECHOs. Improvements in ECG and echocardiographic normative standards, representing age, gender, race, and ethnicity, are needed to increase the efficacy of screening in a young population.
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