LQTS patients’ satisfaction with emergency room care

Abstract: Long QT Syndrome (LQTS) is a genetic condition affecting 1:2,500 that predisposes individuals to cardiac arrhythmias (Ellis et al., 2009). Life threatening symptoms are often unpredictable although there are known triggers to LQTS specific cardiac events. Triggers include physical exertion, emotional upset, and sleep. Events could be triggered by medications, dehydration, and other circumstances that affect cardiac rhythm. Treatments include betablockers, pacemakers, internal cardioverter defibrillators, and automated external defibrillator as precautionary measures. As such, LQTS patients often present to their emergency room (ER) when they become symptomatic. Research suggests that LQTS patients’ experience dissatisfaction in the ER and treatment is a concern (Felgoise et al., 2012). Cardiac training in emergency medicine specialties is limited. Given that ER physician training in cardiology is general, rarer cardiac arrhythmias such as LQTS are unknown by many ER physicians. Studies of ER patient satisfaction have shown that quality of communication and interactions between ER physician and patient, understanding of care, and wait times are related to patient satisfaction in pediatric and adult populations (Margaret et al., 2002). This study aims to learn why LQTS patients are dissatisfied by assessing doctor-patient relationship variables, patient satisfaction, patients’ perception of physicians’ knowledge, and problem-solving skills. The goal is to identify how to increase patients’ ability to be better consumers of ER care, use problem-solving strategies and advocate for appropriate treatment with regard to LQTS-related needs. This study fuses multidisciplinary perspectives for enhancement of patient satisfaction. This is a prospective cross-sectional study of pediatric and adult patients with LQTS and parent perceptions of ER care. A convenience sample of LQTS patients or their parent will complete an online survey. 150 participants will be recruited via announcements on websites focused on cardiac arrhythmias. Measures include Working Alliance Inventory, Personal Information Questionnaire, a standardized ER patient satisfaction questionnaire, and the Social Problem-Solving Inventory. Pearson product moment correlations and multiple regression analyses will be conducted to determine the relationship among the variables. Subscales of the WAI, average estimated wait times, problem-solving skills, and ratings of knowledge and communication from the and ER satisfaction questionnaire will be input as predictor variables and global patient satisfaction will be the criterion variable. Results will lead to understanding ER experiences that may be advantaged by introducing CBT strategies to promote better consumerism of services by LQTS patients. Data will provide physicians with an understanding of LQTS patients’ perceptions of ER care. Findings will determine what predicts satisfaction and whether patients have realistic expectations for ER care. Problem-solving skills training have been effective for helping other medical patients navigate the healthcare system (Nezu et al., 1998), and may be appropriate for LQTS patients. Results will provide guidance for cardiologists and psychologists to best prepare LQTS patients for ER experiences

QoL in Child LQTS Patients Compared to Cardiac Patients

Introduction: • Long QT Syndrome (LQTS) is a life threatening geneticallyinherited cardiac arrhythmia disorder affecting approximately 1:2500 persons1, often diagnosed in childhood. • Management of LQTS changes patients’ lifestyles which can affect quality of life (QoL). Patients have restrictions in physical activity, diet, treatment of noncardiac conditions; take daily doses of medicine and/or have implantable cardiac devices (pacemaker/defibrillator). • General pediatirc cardiac patients show significantly worse QoL in comparison to healthy controls2,3. • Nearly 1 in 5 with other cardiac disorders reported impaired psychosocial functioning3. • The effects of implanted cardiac rhythm devices in cardiac patients demonstrated a significant effect on QoL in pediatric patient scores4. • LQTS patients are typically not assessed for psychological symptoms. • Little research on QoL in pediatric LQTS patients has been conducted to determine if psychosocial interventions are warranted

Ventricular tachycardia in infants with structurally normal heart: A benign disorder

We evaluated the presentation, treatment, and outcome of infants who present with ventricular tachycardia in the first year of life. Seventy-six infants were admitted to our institution with a diagnosis of ventricular tachycardia between January, 1987 and May, 2006. Forty-five infants were excluded from the study because of additional confounding diagnoses including accelerated idioventricular rhythm, Wolff– Parkinson–White syndrome, supraventricular tachycardia with aberrancy, long QT syndrome, cardiac rhabdomyoma, myocarditis, congenital lesions, or incomplete data. The remaining 31 included infants who had a median age at presentation of 1 day, with a range from 1 to 255 days, and a mean ventricular tachycardia rate of 213 beats per minute, with a range from 171 to 280, at presentation. The infants were treated chronically with propranolol (38.7%), amiodarone (12.9%), mexiletine (3.2%), propranolol and mexiletine (9.7%), or propranolol and procainamide (6.5%). The median duration of treatment was 13 months, with a range from 3 to 105 months. Ventricular tachycardia resolved spontaneously in all infants. No patient died, or received catheter ablation or device therapy. Median age at last ventricular tachycardia was 59 days, with a range from 1 to 836 days. Mean follow-up was 45 months, with a range from 5 to 164 months, with a mean ventricular tachycardia-free period of 40 months. Infants with asymptomatic ventricular tachycardia, a structurally normal heart, and no additional electrophysiological diagnosis all had spontaneous resolution of tachycardia. Furthermore, log-rank analysis of the time to ventricular tachycardia resolution showed no difference between children who received chronic outpatient anti-arrhythmic treatment and those who had no such therapy. While indications for therapy cannot be determined from this study, lack of symptoms or myocardial dysfunction suggests that therapy may not be necessary

Pediatric pacemaker infections: Twenty years of experience

AbstractObjective: We sought to evaluate possible predictors of early and late pacemaker infections in children. Methods: A review was performed of all pacemakers implanted in children at The Children's Hospital of Philadelphia between 1982 and 2001. Infections were classified as superficial cellulitus, deep pacemaker pocket infection necessitating removal, or positive blood culture without an identifiable source. Results: A total of 385 pacemakers (224 epicardial and 161 endocardial) were implanted in 267 patients at 8.4 ± 6.2 years. All 2141 outpatient visits were reviewed (median follow-up, 29.4 months; range, 2-232 months). There were 30 (7.8%) pacemaker infections: 19 (4.9%) superficial infections; 9 (2.3%) pocket infections; and 2 (0.5%) isolated positive blood cultures. All superficial infections resolved with intravenous antibiotics. The median time from implantation to infection was 16 days (range, 2 days-5 years). Only 1 deep infection occurred after primary pacemaker implantation. Six patients with deep infections were pacemaker dependent and were successfully managed with intravenous antibiotics, followed by lead-generator removal and implantation of a new pacemaker in a remote location. In univariate analyses trisomy 21 (relative risk, 3.9; P <.01), pacemaker revisions (relative risk, 2.5; P <.01), and single-chamber devices (relative risk, 2.4; P <.05) were identified as predictors of infection. However, in multivariate analyses only trisomy 21 and pacemaker revisions were predictors. Conclusions: The incidences of superficial and deep pacemaker infections were 4.9% and 2.3%, respectively. Trisomy 21 and pacemaker revisions were significant risk factors in the development of infection after pacemaker implantation. For primary pacemaker implantation, the risk of infection requiring system removal is low (0.3%).J Thorac Cardiovasc Surg 2002;124:821-

International criteria for electrocardiographic interpretation in athletes: Consensus statement.

Sudden cardiac death (SCD) is the leading cause of mortality in athletes during sport. A variety of mostly hereditary, structural or electrical cardiac disorders are associated with SCD in young athletes, the majority of which can be identified or suggested by abnormalities on a resting 12-lead electrocardiogram (ECG). Whether used for diagnostic or screening purposes, physicians responsible for the cardiovascular care of athletes should be knowledgeable and competent in ECG interpretation in athletes. However, in most countries a shortage of physician expertise limits wider application of the ECG in the care of the athlete. A critical need exists for physician education in modern ECG interpretation that distinguishes normal physiological adaptations in athletes from distinctly abnormal findings suggestive of underlying pathology. Since the original 2010 European Society of Cardiology recommendations for ECG interpretation in athletes, ECG standards have evolved quickly, advanced by a growing body of scientific data and investigations that both examine proposed criteria sets and establish new evidence to guide refinements. On 26-27 February 2015, an international group of experts in sports cardiology, inherited cardiac disease, and sports medicine convened in Seattle, Washington (USA), to update contemporary standards for ECG interpretation in athletes. The objective of the meeting was to define and revise ECG interpretation standards based on new and emerging research and to develop a clear guide to the proper evaluation of ECG abnormalities in athletes. This statement represents an international consensus for ECG interpretation in athletes and provides expert opinion-based recommendations linking specific ECG abnormalities and the secondary evaluation for conditions associated with SCD

International criteria for electrocardiographic interpretation in athletes: Consensus statement

Sudden cardiac death (SCD) is the leading cause of mortality in athletes during sport. A variety of mostly hereditary, structural or electrical cardiac disorders are associated with SCD in young athletes, the majority of which can be identified or suggested by abnormalities on a resting 12-lead electrocardiogram (ECG). Whether used for diagnostic or screening purposes, physicians responsible for the cardiovascular care of athletes should be knowledgeable and competent in ECG interpretation in athletes. However, in most countries a shortage of physician expertise limits wider application of the ECG in the care of the athlete. A critical need exists for physician education in modern ECG interpretation that distinguishes normal physiological adaptations in athletes from distinctly abnormal findings suggestive of underlying pathology. Since the original 2010 European Society of Cardiology recommendations for ECG interpretation in athletes, ECG standards have evolved quickly, advanced by a growing body of scientific data and investigations that both examine proposed criteria sets and establish new evidence to guide refinements. On 26-27 February 2015, an international group of experts in sports cardiology, inherited cardiac disease, and sports medicine convened in Seattle, Washington (USA), to update contemporary standards for ECG interpretation in athletes. The objective of the meeting was to define and revise ECG interpretation standards based on new and emerging research and to develop a clear guide to the proper evaluation of ECG abnormalities in athletes. This statement represents an international consensus for ECG interpretation in athletes and provides expert opinion-based recommendations linking specific ECG abnormalities and the secondary evaluation for conditions associated with SCD

Clinical Sequencing Exploratory Research Consortium: Accelerating Evidence-Based Practice of Genomic Medicine

Despite rapid technical progress and demonstrable effectiveness for some types of diagnosis and therapy, much remains to be learned about clinical genome and exome sequencing (CGES) and its role within the practice of medicine. The Clinical Sequencing Exploratory Research (CSER) consortium includes 18 extramural research projects, one National Human Genome Research Institute (NHGRI) intramural project, and a coordinating center funded by the NHGRI and National Cancer Institute. The consortium is exploring analytic and clinical validity and utility, as well as the ethical, legal, and social implications of sequencing via multidisciplinary approaches; it has thus far recruited 5,577 participants across a spectrum of symptomatic and healthy children and adults by utilizing both germline and cancer sequencing. The CSER consortium is analyzing data and creating publically available procedures and tools related to participant preferences and consent, variant classification, disclosure and management of primary and secondary findings, health outcomes, and integration with electronic health records. Future research directions will refine measures of clinical utility of CGES in both germline and somatic testing, evaluate the use of CGES for screening in healthy individuals, explore the penetrance of pathogenic variants through extensive phenotyping, reduce discordances in public databases of genes and variants, examine social and ethnic disparities in the provision of genomics services, explore regulatory issues, and estimate the value and downstream costs of sequencing. The CSER consortium has established a shared community of research sites by using diverse approaches to pursue the evidence-based development of best practices in genomic medicine