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INTRODUCTION/AIMS: Type 1 myotonic dystrophy (DM1) is a neuromuscular disorder of multiple organ systems with important electrophysiologic (EP) manifestations, leading to a cumulative incidence of sudden death of 6.6%. Due to genetic anticipation, there is a pediatric subset of this patient population. However, most EP research on DM1 patients has been in adults, making cardiac care for pediatric patients difficult and directed by adult guidelines which often leads to cardiovascular implantable electronic device (CIED) implants. We sought to investigate the prevalence of CIEDs in the pediatric DM1 population. METHODS: The Vizient® Clinical Data Base was queried from October 2019 to October 2023 for admissions with and without ICD-10 code for myotonic dystrophy (G71.11), with and without codes for presence of a pacemaker or ICD (Z95.0, Z95.810). Patients who were identified were stratified by age: Pediatric (0-21 years) and Adult (22-50 years). RESULTS: Prevalence of CIED in pediatric DM1 was 2.1% and in adult DM1 was 15.8%. When comparing to pediatric and adult patients with CIED and without DM1, the odds ratio for CIED in pediatric DM1 was 48.8, compared to 23.3 for CIED in adult DM1. DISCUSSION: There are pediatric DM1 patients who have received CIED despite a lack of data to inform this decision-making. Further research will be important to ensure appropriate use of CIED in this population and to develop appropriate guidelines to direct management.
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Patients with Fontan physiology have reduced exercise performance compared to their peers as well as a higher incidence of bundle branch block (BBB). This study aims to investigate the association between BBB and exercise performance in the Fontan population through a retrospective review of the Pediatric Heart Network Fontan study public use dataset. "Low Performers" were defined as ≤ 25th percentile (for Fontan patients) for each exercise parameter at anaerobic threshold (AT) for gender and age and "Normal Performers" were all other patients. A total of 303 patients with Fontan physiology who underwent exercise testing reached AT and had complete data for BBB. BBB occurred more frequently in Low Performers for VO2 [OR (95% CI): 2.6 (1.4, 4.8)] and Work [OR (95% CI): 2.7 (1.4, 5.1)], suggesting that BBB in the Fontan population is associated with reduced exercise performance. This data adds to the existing clinical evidence of the adverse effects of conduction abnormalities on single ventricle cardiac output and adds support for consideration of cardiac resynchronization and multi-site ventricular pacing in this patient population.
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INTRODUCTION: Accessory atrioventricular pathways (APs) may mediate atrioventricular reciprocating tachycardia and, in some cases, have the potential to conduct atrial tachycardia rapidly, which can be life threatening. While catheter ablation can be curative, ablation of right free wall APs is associated with a high rate of recurrence, likely secondary to reduced catheter stability along the right free wall atrioventricular groove. We sought to identify characteristics associated with a lower rate of recurrence and hypothesized ablation lesions placed on the ventricular side of the atrioventricular groove using a retroflexed catheter approach would decrease rates of recurrence. METHODS AND RESULTS: Retrospective chart review of patients who underwent catheter ablation of a right free wall AP from January 1, 2008 through June 1, 2021 with >2 months follow up. Cox proportional hazards regression was used to identify relationships between predictor variables and AP recurrence. We identified 95 patients who underwent ablation of 98 right free wall APs. Median age was 13.1 years and median weight at ablation was 52.3 kg. Overall, 23/98 (23%) APs recurred. Use of a retroflexed catheter course approaching the atrioventricular groove from the ventricular aspect was associated with reduced risk of AP recurrence with (univariable hazard ratio of 0.10 [95% confidence interval: 0.01-0.78]), which remained significant in multiple two variable Cox proportional hazards models. CONCLUSION: Use of a retroflexed catheter course is associated with a reduced likelihood of AP recurrence. This approach results in improved catheter stability and should be considered for ablation of right free wall APs.
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Feixe Acessório Atrioventricular , Ablação por Cateter , Septo Interventricular , Síndrome de Wolff-Parkinson-White , Humanos , Adolescente , Estudos Retrospectivos , Fascículo Atrioventricular , Feixe Acessório Atrioventricular/cirurgia , Catéteres , Ablação por Cateter/efeitos adversosRESUMO
Regular wide complex tachycardia carries with it a standard array of differential diagnoses. This electrocardiogram demonstrates wide complex tachycardia and multiple QRS configurations in a neonate without structural heart disease with an uncommon suspected underlying diagnosis. (Level of Difficulty: Advanced.).
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INTRODUCTION: Guidelines recommend trans-esophageal echocardiography (TEE) for patients with atrial fibrillation (AF) or atrial flutter (AFL) for >48 h, due to risk of intracardiac thrombus formation. With growing evidence that AFL in adults with structurally normal hearts has less thrombogenic potential compared to AF, and the need for TEE questioned, we compared prevalence of intracardiac thrombus detected by TEE in pediatric and congenital heart disease (CHD) patients presenting in AF and AFL. METHODS/RESULTS: Single-center, cross-sectional analysis for unique first-time presentations of patients for either AF, AFL, or intra-atrial reentrant tachycardia (IART) between 2000 and 2019. Patients were categorized by presenting arrhythmia (AF vs. AFL/IART), with the exclusion of other forms of atrial tachycardia, hemodynamic instability, chronic anti-coagulation before TEE, and presentation for a reason other than TEE examination for thrombus. A total of 201 patients had TEE with co-diagnosis of AF or AFL. Of these, 105 patients (29 AF, 76 AFL) met inclusion criteria, with no difference in age between AF (median 24.9 years; IQR 18.6-38.3 years) and AFL/IART (23.3 years; 15.4-38.4 years). The prevalence of thrombus in the entire cohort was 9.5%, with no difference between AF (13.8%) and AFL groups (7.9%), p = .46. Patients with thrombus demonstrated no difference in age, systemic ventricular function, cardiac complexity, or CHADS2/CHA2DS2VASc score at presentation. CONCLUSIONS: The risk for intracardiac thrombus is high in the pediatric and CHD population, with no apparent distinguishing factors to warrant a change in the recommendations for TEE, with all levels of cardiac complexity being at risk for clot.
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Fibrilação Atrial , Flutter Atrial , Cardiopatias Congênitas , Trombose , Adolescente , Adulto , Fibrilação Atrial/diagnóstico por imagem , Fibrilação Atrial/epidemiologia , Flutter Atrial/diagnóstico por imagem , Flutter Atrial/epidemiologia , Criança , Estudos Transversais , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/epidemiologia , Humanos , Prevalência , Trombose/diagnóstico por imagem , Trombose/epidemiologia , Adulto JovemRESUMO
OBJECTIVE: This study evaluates the ability of experienced pediatric electrophysiologists (EPs) to reliably classify incomplete right bundle branch block (IRBBB) and assesses its clinical utility as an isolated ECG finding in a group of healthy outpatient children without prior cardiac evaluation. DESIGN: We performed a retrospective analysis of all electrocardiographic and echocardiographic records at Boston Children's Hospital between January 1, 2005, and December 31, 2014. Echocardiographic diagnoses were identified if registered between the date of the index electrocardiogram and the ensuing year. A selected subset of 473 ECGs was subsequently reanalyzed in a blinded manner by six pediatric EPs to determine the consistency with which the finding of IRBBB could be assigned. RESULTS: Of the 331 278 ECGs registered in the BCH database, 32 127 (9.7%) met inclusion criteria and were analyzed for the prevalence of isolated right bundle conduction disturbance findings. The mean age was 12.1 ± 4.0 years, and the population was 49% male. Of the 32 127 ECGs, 72.5% were coded normal, 3.0% were coded IRBBB, and 0.5% were coded complete right bundle branch block (CRBBB). A total of 7.3% of patients coded as normal had an ensuing echocardiogram, compared to 12.5% coded IRBBB. Echo findings were recorded in 0.1% of normal and 0.2% of IRBBB. Patients with ASD-secundum type were no more likely to have isolated IRBBB on previous ECG than the general population (2.5% vs 3.0%). Analysis of inter-reader variability in ECG findings and conduction disturbance identification was high (range of IRBBB prevalence 1-20% among readers). Reinterpretation of ECGs using explicit diagnostic criteria did not demonstrate consistent discrimination of IRBBB and Normal ECGs. CONCLUSIONS: IRBBB is not uncommon in a healthy school age population and is observed to have high inter-reader variability. It was associated with increased use of echocardiographic exam but was not associated with increased rate of echocardiographic findings when compared with rates for normal ECGs.
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Bloqueio de Ramo/fisiopatologia , Eletrocardiografia/métodos , Sistema de Condução Cardíaco/fisiopatologia , Monitorização Fisiológica/métodos , Adolescente , Boston/epidemiologia , Bloqueio de Ramo/diagnóstico , Bloqueio de Ramo/epidemiologia , Criança , Pré-Escolar , Ecocardiografia , Feminino , Seguimentos , Humanos , Masculino , Prevalência , Estudos RetrospectivosRESUMO
Adults with congenital heart disease (CHD) are a rapidly increasing population and their impact on healthcare resources is not fully understood. The purpose of this study was to describe the costs of hospitalizations for non-cardiac disease for adults with CHD. We conducted a retrospective review of hospital discharge data from the University HealthSystem Consortium Clinical Data Base/Resource Manager from January 2011 through December 2013. Patients were ≥ 18 years old at admission with any ICD-9 code for moderate or high severity CHD; cardiac surgical admissions were excluded. The comparison group consisted of patients ≥ 18 years old with no ICD-9 codes for any severity CHD. There were 9,169,700 non-CHD, 28,224 moderate CHD, and 3045 high severity CHD hospital admissions. Total length of stay was longer for acute kidney injury, depressive disorder, esophageal reflux, and obstructive sleep apnea for any severity CHD; ICU admission rates were higher for all diagnoses with any severity CHD. Mean observed direct costs were higher for all diagnoses for moderate CHD and all diagnoses except dehydration, type 2 diabetes, obesity, and obstructive sleep apnea for high severity CHD. This review identified significantly increased hospitalization costs for adults with moderate and high severity CHD who are admitted for non-cardiac medical conditions not associated with concomitant cardiac surgical procedures. Admissions with CHD diagnoses had higher ICU admission rates, longer lengths of stay, and higher mortality for most non-cardiac admission diagnoses. These data will add to our understanding of the economic impact of adults with CHD.
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Cardiopatias Congênitas/economia , Preços Hospitalares/estatística & dados numéricos , Hospitalização/economia , Adolescente , Adulto , Idoso , Bases de Dados Factuais , Feminino , Recursos em Saúde , Cardiopatias Congênitas/epidemiologia , Cardiopatias Congênitas/mortalidade , Mortalidade Hospitalar , Humanos , Tempo de Internação/estatística & dados numéricos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Adulto JovemRESUMO
Sudden cardiac arrest is a rare but devastating cause of death in young adults. Electrocardiograms may detect many causes of sudden cardiac arrest, but are not routinely included in pre-athletic screening in the United States of America partly because of high rates of false-positive interpretation. To improve electrocardiogram specificity for identifying cardiac conditions associated with sudden cardiac arrest, an expert panel developed refined criteria known as the Seattle Criteria. Ours is the first study to compare standard electrocardiogram criteria with Seattle Criteria in 11- to 13-year-olds. In total, 1424 students completed the pre-athletic screening and electrocardiogram; those with a positive screen or abnormal electrocardiogram interpreted by a paediatric electrophysiologist completed further work-up. Electrocardiograms referred for additional evaluation were re-interpreted by a paediatric electrophysiologist using Seattle Criteria. Electrocardiogram abnormalities were identified in 98 (6.9%); Seattle Criteria identified 28 (2.0%). Formal evaluation confirmed four students at risk for sudden cardiac arrest (0.3%): long QT syndrome (n=2), Wolff-Parkinson-White (n=1), and pulmonary hypertension (n=1). All students with at-risk phenotypes for sudden cardiac arrest were identified by both standard electrophysiologist and Seattle Criteria. The false-positive interpretation rate decreased from 6.6 to 1.7% with Seattle Criteria. Downstream costs associated with screening using standard paediatric electrocardiogram interpretations and Seattle Criteria were projected at $24 versus $7, respectively. In conclusion, using Seattle Criteria for electrocardiogram interpretation decreases the rate of false-positive results compared with standard interpretation without omitting true-positive electrocardiogram findings. This may decrease unnecessary referrals and costs associated with formal cardiology evaluation.
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Atletas , Morte Súbita Cardíaca/prevenção & controle , Eletrocardiografia/métodos , Cardiopatias/diagnóstico , Programas de Rastreamento/métodos , Adolescente , Causas de Morte/tendências , Criança , Morte Súbita Cardíaca/epidemiologia , Morte Súbita Cardíaca/etiologia , Reações Falso-Positivas , Feminino , Cardiopatias/complicações , Humanos , Masculino , Estudos Retrospectivos , Estudantes , Taxa de Sobrevida/tendências , Estados Unidos/epidemiologiaRESUMO
Single ventricle congenital heart disease (SV CHD) has transformed from a nearly universally fatal condition to a chronic illness. As the number of adults living with SV CHD continues to increase, there needs to be an understanding of health care resource utilization (HCRU), particularly for noncardiac conditions, for this patient population. We performed a retrospective database review of the University HealthSystem Consortium Clinical Database/Resource Manager for adult patients with SV CHD hospitalized for noncardiac conditions from January 2011 to November 2014. Patients with SV CHD were identified using International Classification of Disease (ICD)-9 codes associated with SV CHD (hypoplastic left heart, tricuspid atresia, and SV) and stratified into 2 groups by age (18 to 29 years and 30 to 40 years). Direct cost, length of stay (LOS), intensive care unit (ICU) admission rate and mortality data were compared with age-matched patients without CHD. There were 2,083,651 non-CHD and 590 SV CHD admissions in Group 1 and 2,131,046 non-CHD and 297 SV CHD admissions in Group 2. There was no difference in LOS in Group 1, but there were higher costs for several diagnoses. LOS and costs were higher for several diagnoses in Group 2. ICU admission rate and in-hospital mortality were higher for several diagnoses for patients with SV CHD in both groups. In conclusion, adults with SV CHD admitted for noncardiac diagnoses have higher HCRU (longer LOS and higher ICU admission rates) compared with similarly aged patients without CHD. These findings stress the importance of good primary care in this population with complex, chronic cardiac disease to prevent hospitalizations and higher HCRU.
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Cardiopatias Congênitas/epidemiologia , Ventrículos do Coração/anormalidades , Hospitalização/estatística & dados numéricos , Sobreviventes , Adolescente , Adulto , Comorbidade , Cardiopatias Congênitas/mortalidade , Custos Hospitalares , Hospitalização/economia , Humanos , Unidades de Terapia Intensiva/estatística & dados numéricos , Tempo de Internação , Estudos Retrospectivos , Adulto JovemRESUMO
BACKGROUND: Recent studies have demonstrated higher-quality chest compressions (CCs) following a 60 s ultra-brief video (UBV) on compression-only CPR (CO-CPR). However, the effectiveness of UBVs as a CPR-teaching tool for lay bystanders in public venues remains unknown. OBJECTIVE: Determine whether an UBV is effective in teaching laypersons CO-CPR in a public setting and if viewing leads to superior responsiveness and CPR skills. METHODS: Adult lay bystanders were enrolled in a public shopping mall and randomized to: (1) Control (CTR): sat idle for 60 s; (2) UBV: watched a 60 s UBV on CO-CPR. Subjects were read a scenario detailing a sudden collapse in the mall and asked to do what they "thought was best" on a mannequin. Performance measures were recorded for 2 min: responsiveness (time to call 911 and first CCs) and CPR quality [CC depth, rate, hands-off interval (time without CC after first CC)]. RESULTS: One hundred subjects were enrolled. Demographics were similar between groups. UBV subjects called 911 more frequently (percent difference: 31%) and initiated CCs sooner in the arrest scenario (median difference (MD): 5 s). UBV cohort had increased CC rate (MD: 19 cpm) and decreased hands-off interval (MD: 27 s). There was no difference in CC depth. CONCLUSION: Bystanders with UBV training in a shopping mall had significantly improved responsiveness, CC rate, and decreased hands-off interval. Given the short length of training, UBV may have potential as a ubiquitous intervention for public venues to help improve bystander reaction to arrest and CO-CPR performance.
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Reanimação Cardiopulmonar/educação , Reanimação Cardiopulmonar/métodos , Parada Cardíaca Extra-Hospitalar/terapia , Gravação em Vídeo , Adulto , Feminino , Humanos , Masculino , Tórax , Fatores de TempoRESUMO
BACKGROUND: It is not known whether prisoners of war (POWs) are more likely to develop dementia independently of the effects of posttraumatic stress disorder (PTSD). METHODS: We performed a retrospective cohort study in 182,879 U.S. veterans age 55 years and older, and examined associations between POW status and PTSD at baseline (October 1, 2000-September 30, 2003), and incident dementia during follow-up (October 1, 2003-September 30, 2012). RESULTS: A total of 484 veterans (0.3%) reported being POWs, of whom 150 (31.0%) also had PTSD. After adjusting for demographics, medical and psychiatric comorbidities, period of service, and the competing risk of death, the risk of dementia was increased in veterans who were POWs only (hazard ratio [HR], 1.61; 95% confidence interval [CI], 1.30-1.98) or had PTSD only (HR, 1.52; 95% CI, 1.41-1.64) and was greatest in veterans who were POWs and also had PTSD (HR, 2.24; 95% CI, 1.72-2.92). CONCLUSIONS: POW status and PTSD increase risk of dementia in an independent, additive manner in older veterans.