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1.
J Pediatr ; : 114145, 2024 Jun 13.
Artigo em Inglês | MEDLINE | ID: mdl-38878963

RESUMO

OBJECTIVE: To determine the long-term outcomes among a cohort of patients with Kawasaki disease (KD) and a history of giant coronary artery aneurysms (CAA) at a single US center. RESULTS: There were 60 patients with KD and giant CAAs identified between 1989 and 2023. The majority of patients were male (71.7%) with median age at diagnosis of 0.9 years (0.2-13.3). Patients were followed for a median of 11 years, up to 34.5 years. MACE occurred in 13 (21.7%) patients at a median of 1.4 years (0.04-22.6) after KD diagnosis. The 10-, 20-, and 30-year MACE-free rates were 75%, 75%, and 60%. Patients with maximal CA z-scores ≥20 or bilateral CAA were more likely to have MACE. During follow-up, 26.7% of CAA regressed to normal luminal diameter at a median of 3.6 years (0.6-12.0). The 10-, 20- and 30-year likelihood of CA regression to normal luminal diameter was 36%, 46%, and 46%. CONCLUSIONS: Over 30 years, MACE occurred in nearly 22% of patients, more often in those with bilateral CAA or CA z-scores ≥20. Despite regression to normal luminal diameter in over 25% of CAA, patients with a history of KD-associated giant CAA require ongoing surveillance for cardiac complications, even years after the initial disease.

2.
Pediatr Cardiol ; 2023 Aug 06.
Artigo em Inglês | MEDLINE | ID: mdl-37544951

RESUMO

BACKGROUND: Published guidelines provide recommendations for risk stratification in pediatric Wolff-Parkinson-White (WPW). There are no data on provider concordance with these guidelines. We hypothesized that significant practice variation exists between pediatric cardiologists (PC) and electrophysiologists (EP). METHOD: The records of all patients, age 8 to 21 years, with a new ECG diagnosis of WPW between 1/1/2013 and 12/31/2018, from a single center, were retrospectively reviewed. Subjects were categorized on the basis of symptoms and resting ECG findings as one of the following: asymptomatic intermittent WPW, asymptomatic persistent WPW, or symptomatic WPW. The performance and results of diagnostic testing, including Holter monitor, event monitor, exercise stress test (EST), and electrophysiology study (EPS), were recorded. The primary outcome was concordance with published guidelines. A secondary outcome was documentation of a discussion of sudden cardiac death (SCD) risk. RESULTS: 615 patient encounters were analyzed in 231 patients with newly diagnosed WPW pattern on ECG (56% male; mean age at diagnosis 13.9 ± 2.5 years). EP were observed to have a significantly higher rate of guideline concordance than PC (95% vs. 71%, p < 0.001). There was significant practice variation between PC and EP in the documentation of a discussion of SCD risk: 96% in EP vs. 39% in PC (p < 0.001). CONCLUSION: Significant practice variation exists in the non-invasive and invasive risk stratification of pediatric WPW, with lower concordance to published guidelines amongst PC, when compared to EP. This report highlights the need to promote awareness of current WPW guidelines in the pediatric cardiology community at large.

3.
Pediatr Cardiol ; 2023 Sep 19.
Artigo em Inglês | MEDLINE | ID: mdl-37725123

RESUMO

In children with hypertrophic cardiomyopathy (HCM), the genotype-phenotype association of abnormal electrocardiographic (ECG) features in the backdrop of gene positivity has not been well described. This study aimed to describe the abnormal ECG findings in children with HCM harboring who have genetic variants and determine the association with major adverse cardiac events (MACE). We retrospectively analyzed 81 variants-positive, phenotype-positive (V+P+), 66 variant-positive, phenotype-negative (V+P-), and 85 non-sarcomeric subjects. We analyzed ECG findings and clinical outcomes in the three groups of subjects. Repolarization abnormalities (ST and T wave changes) and pathologic Q waves were the most common abnormalities in variant and non-sarcomeric subjects. The V+P+ group showed higher occurrence of ST segment changes and T wave abnormalities compared to V+P- group. Independent predictors of MACE included ST segment changes (OR 3.54, CI 1.20-10.47, p = 0.022). T wave changes alone did not predict outcome (OR 2.13, CI 0.75-6.07, p = 0.157), but combined repolarization abnormalities (ST+T changes) were strong predictors of MACE (OR 5.84, CI 1.43-23.7, p = 0.014) than ST segment changes alone. Maximal wall z score by echocardiography was a predictor of MACE (OR 1.21, CI 1.07-1.37, p = 0.002). Despite the presence of significant myocardial hypertrophy (z score > 4.7), voltage criteria for LVH were much less predictive. In the non-sarcomeric group, RVH was significantly associated with MACE (OR 3.85, CI 1.08-13.73, p = 0.038). These abnormal ECG findings described on the platform of known genetic status and known myocardial hypertrophy may add incremental value to the diagnosis and surveillance of disease progression in children with HCM. Select ECG findings, particularly repolarization abnormalities, may serve as predictors of MACE in children.

4.
J Pediatr ; 206: 113-118, 2019 03.
Artigo em Inglês | MEDLINE | ID: mdl-30573213

RESUMO

OBJECTIVES: To assess the impact of participation in an educational presentation on electrocardiogram (ECG) interpretation in children on pediatric practitioners' ability to accurately interpret ECGs. STUDY DESIGN: Pediatric healthcare providers at a pediatric clinic with >65 000 visits/year were eligible to participate. A 1-hour ECG educational module that provided a systematic approach to ECG interpretation was presented to 8 providers who consented (6 pediatricians, 2 pediatric nurse practitioners). A test on 11 ECGs (normal, normal-variant, and abnormal ECGs) was given before and 2 weeks after the educational module. Outcomes included correct interpretation of each ECG as normal or abnormal and correct identification of specific ECG findings. Data analysis was descriptive and included χ2 and Student t test. RESULTS: Mean score (SD) for correct interpretation of ECGs as normal or abnormal improved from 35% (48%) (95% CI 25.0-45.4) to 77% (42%) (95% CI 68.3-86.2) after the ECG educational module (P < .001). Mean (SD) pretest score for correct identification in the normal ECG category improved from 45% (50%) (95% CI 28.9-61.1) to 68% (47%) (95% CI 52.3-82.7) (P= .003). In the abnormal ECG category, correct identification improved from 31% (47%) (95% CI 17.6-44.9) to 83% (5%) (95% CI 72.4-94.3) after the module (P < .001). CONCLUSIONS: Education of pediatric practitioners on ECG interpretation significantly improves their ability to distinguish normal from abnormal and to identify specific abnormalities. Limitations included small sample size and short-term follow-up.


Assuntos
Cardiologia/métodos , Cardiologia/normas , Eletrocardiografia , Pediatria , Arritmias Cardíacas/diagnóstico , Criança , Competência Clínica , Morte Súbita Cardíaca/prevenção & controle , Estudos de Viabilidade , Feminino , Humanos , Capacitação em Serviço , Masculino , Variações Dependentes do Observador , Profissionais de Enfermagem Pediátrica , Estudos Prospectivos , Reprodutibilidade dos Testes
5.
Eur Heart J ; 39(16): 1466-1480, 2018 04 21.
Artigo em Inglês | MEDLINE | ID: mdl-28329355

RESUMO

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 over the last decade; pushed by a growing body of scientific data that both tests proposed criteria sets and establishes 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, 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.


Assuntos
Atletas , Eletrocardiografia , Coração/fisiologia , Morte Súbita Cardíaca/prevenção & controle , Eletrocardiografia/normas , Coração/fisiopatologia , Cardiopatias/diagnóstico , Cardiopatias/fisiopatologia , Humanos
6.
Br J Sports Med ; 51(9): 704-731, 2017 May.
Artigo em Inglês | MEDLINE | ID: mdl-28258178

RESUMO

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.


Assuntos
Morte Súbita Cardíaca/prevenção & controle , Eletrocardiografia/normas , Cardiopatias/diagnóstico , Medicina Esportiva/normas , Adolescente , Adulto , Atletas , Criança , Consenso , Humanos , Programas de Rastreamento , Washington , Adulto Jovem
7.
Hum Genomics ; 9: 15, 2015 Jul 19.
Artigo em Inglês | MEDLINE | ID: mdl-26187847

RESUMO

BACKGROUND: Conditions associated with sudden cardiac arrest/death (SCA/D) in youth often have a genetic etiology. While SCA/D is uncommon, a pro-active family screening approach may identify these inherited structural and electrical abnormalities prior to symptomatic events and allow appropriate surveillance and treatment. This study investigated the diagnostic utility of exome sequencing (ES) by evaluating the capture and coverage of genes related to SCA/D. METHODS: Samples from 102 individuals (13 with known molecular etiologies for SCA/D, 30 individuals without known molecular etiologies for SCA/D and 59 with other conditions) were analyzed following exome capture and sequencing at an average read depth of 100X. Reads were mapped to human genome GRCh37 using Novoalign, and post-processing and analysis was done using Picard and GATK. A total of 103 genes (2,190 exons) related to SCA/D were used as a primary filter. An additional 100 random variants within the targeted genes associated with SCA/D were also selected and evaluated for depth of sequencing and coverage. Although the primary objective was to evaluate the adequacy of depth of sequencing and coverage of targeted SCA/D genes and not for primary diagnosis, all patients who had SCA/D (known or unknown molecular etiologies) were evaluated with the project's variant analysis pipeline to determine if the molecular etiologies could be successfully identified. RESULTS: The majority of exons (97.6 %) were captured and fully covered on average at minimum of 20x sequencing depth. The proportion of unique genomic positions reported within poorly covered exons remained small (4 %). Exonic regions with less coverage reflect the need to enrich these areas to improve coverage. Despite limitations in coverage, we identified 100 % of cases with a prior known molecular etiology for SCA/D, and analysis of an additional 30 individuals with SCA/D but no known molecular etiology revealed a diagnostic answer in 5/30 (17 %). We also demonstrated 95 % of 100 randomly selected reported variants within our targeted genes would have been picked up on ES based on our coverage analysis. CONCLUSIONS: ES is a helpful clinical diagnostic tool for SCA/D given its potential to successfully identify a molecular diagnosis, but clinicians should be aware of limitations of available platforms from technical and diagnostic perspectives.


Assuntos
Morte Súbita Cardíaca , Exoma/genética , Genômica , Sequenciamento de Nucleotídeos em Larga Escala/métodos , Adolescente , Alelos , Criança , Genoma Humano , Humanos , Análise de Sequência de DNA , Adulto Jovem
8.
Pediatr Cardiol ; 37(4): 722-7, 2016 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-26717911

RESUMO

In the outpatient setting, children who present with syncope routinely undergo electrocardiograms (ECG). Because of concerns for hypertrophic cardiomyopathy, children with syncope meeting ECG criteria for left ventricular hypertrophy (LVH) will frequently undergo an echocardiogram. Our objectives were to determine whether Davignon criteria for ECG waves overestimate LVH in children presenting with syncope, and to study the usefulness of echocardiography in these children. We hypothesize that the Davignon criteria presently used for interpretation of ECGs overestimate LVH, resulting in unnecessary echocardiography in this clinical setting. The clinical database of The Children's Hospital of Philadelphia was evaluated from 2002 to 2012 to identify children between 9 and 16 years of age, who presented with non-exercise-induced, isolated syncope. From this group of patients, only those with clear-cut evidence of LVH (by Davignon criteria), who also underwent an echocardiogram, were selected. A total of 136 children with syncope were identified as having LVH by Davignon ECG criteria. None of these patients manifested any evidence of hypertrophic cardiomyopathy, with normal ventricular septum (average Z-score -0.68 ± 0.84), LV posterior wall (average Z-score -0.66 ± 1.18) and LV mass (average Z-score 0.52 ± 1.29). No significant correlation was found between summed RV6 plus SV1 and LV mass. Correlations between additional ECG parameters and measures of LVH by echocardiography were similarly poor. In children presenting with syncope and LVH by ECG, there was no evidence of true LVH by echocardiography. We propose that the Davignon ECG criteria for interpreting LVH in children overestimate the degree of hypertrophy in these children and the yield of echocardiography is extremely low.


Assuntos
Ecocardiografia , Eletrocardiografia/normas , Ventrículos do Coração/fisiopatologia , Hipertrofia Ventricular Esquerda/diagnóstico , Síncope/etiologia , Adolescente , Criança , Feminino , Humanos , Modelos Lineares , Masculino , Philadelphia , Reprodutibilidade dos Testes , Estudos Retrospectivos
9.
Am Heart J ; 169(3): 426-437.e23, 2015 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-25728734

RESUMO

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.


Assuntos
Doenças Cardiovasculares/mortalidade , Mortalidade da Criança , Arritmias Cardíacas/mortalidade , Cardiomiopatias/mortalidade , Causas de Morte , Criança , Mortalidade da Criança/tendências , Pré-Escolar , Feminino , Cardiopatias Congênitas/mortalidade , Humanos , Masculino , Estados Unidos/epidemiologia
10.
J Pediatr ; 217: 218-219, 2020 02.
Artigo em Inglês | MEDLINE | ID: mdl-31732134
11.
J Electrocardiol ; 48(3): 316-23, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-25788105

RESUMO

Screening for conditions associated with sudden cardiac arrest in the United States (US) is aimed at high school athletes in most states and utilizes a preparticipation history and physical form that is not standardized across the US. In Italy, data have shown that their incidence of sudden cardiac arrest has decreased significantly after implementation of an electrocardiographic-based screening program including history and physical exam. The American Heart Association recommendations do not include an electrocardiogram. A recent AHA statement has suggested that those screening athletes should consider all children of similar ages in the selected venue, but still should not include an electrocardiogram. A number of models of screening are presented along with a best practice recommendation for further evaluation and study.


Assuntos
Atletas/classificação , Cardiomiopatias/diagnóstico , Morte Súbita Cardíaca/prevenção & controle , Testes Diagnósticos de Rotina/normas , Eletrocardiografia/normas , Testes Obrigatórios/normas , Cardiomiopatias/prevenção & controle , Europa (Continente) , Humanos , Programas de Rastreamento/normas , Exame Físico/normas , Guias de Prática Clínica como Assunto , Prognóstico , Medicina Esportiva/normas , Washington
12.
Am Heart J ; 168(4): 568-576.e3, 2014 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-25262268

RESUMO

BACKGROUND: The only systematic collection of cardiovascular (CV) deaths in children resides in the database derived from the Case Reporting System of the National Center for the Review and Prevention of Child Deaths (NCRPCD). We describe the process used to develop an analytical data set to inform our understanding of CV deaths in children from this database. METHODS: Twenty-five states reporting natural CV deaths during 2005 to 2009 were contacted. Sixteen states agreed to participate. Cases experienced a natural CV death and were 0 to 21 years. Challenges to building a final analytical data set were identified and included reclassification, recategorization, and the development of new variables from existing data, including an algorithm to identify sudden cardiac deaths. RESULTS: The final data set included 1,098 cases. Missing data comprised a mean of 41.7% for most key variables. 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%; and Hispanic, 19.5%. CONCLUSIONS: This manuscript provides the first description of the natural CV death data set from the NCRPCD. We identify potential beneficial changes in the NCRPCD Case Reporting System and review process. Analysis of these data will help determine characteristics of CV deaths and allow the assessment of risk factors that can be used to prevent CV death in the young. The rate of CV death can be lowered using knowledge of associations that can be gleaned from this robust database. Best practices for prevention hold promise for a future with fewer deaths that will need to be reviewed.


Assuntos
Doenças Cardiovasculares/mortalidade , Programas Governamentais/estatística & dados numéricos , Registros , Sistema de Registros , Adolescente , Causas de Morte/tendências , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Masculino , Estudos Retrospectivos , Fatores de Risco , Taxa de Sobrevida/tendências , Estados Unidos/epidemiologia , Adulto Jovem
13.
Curr Opin Cardiol ; 29(1): 68-75, 2014 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-24284981

RESUMO

PURPOSE OF REVIEW: Over half of young sudden cardiac arrest victims show no prior warning signs or symptoms and survival depends on secondary prevention, notably prompt initiation of cardiopulmonary resuscitation (CPR) and the use of an automated external defibrillator (AED), for out-of-hospital arrests. There is increasing public interest in and uptake of public access defibrillation programs in communities and schools. Our purpose was to review recent data on sudden cardiac arrest in children and its outcome to identify ways to improve the current low survival rate of youth who experience sudden cardiac arrest. RECENT FINDINGS: Increases in bystander cardiopulmonary resuscitation in Norway to 73% have occurred alongside increasing survival from sudden cardiac arrest from shockable rhythms to 52%. Studies in Denmark and the US show that survival of 69-74% is possible when a shockable rhythm is present and an automated external defibrillator is immediately applied. Up to 70-80% of US schools have automated external defibrillators, but not all have effective emergency action plans to maximize the impact of the presence of the AED. SUMMARY: Studies suggest that education to increase bystander CPR and implementation of school AEDs and other public access defibrillation programs improve the survival of youth experiencing sudden cardiac arrest to 74% when optimal programs are in place. Methods to enhance such programs are presented. All involved with the health and education of youth are urged to implement best practices to protect youth and improve survival from sudden cardiac arrest.


Assuntos
Reanimação Cardiopulmonar/métodos , Morte Súbita Cardíaca/prevenção & controle , Desfibriladores , Cardioversão Elétrica/métodos , Parada Cardíaca Extra-Hospitalar/terapia , Prevenção Secundária/métodos , Adolescente , Reanimação Cardiopulmonar/educação , Criança , Pré-Escolar , Educação em Saúde/métodos , Humanos , Lactente , Taxa de Sobrevida , Resultado do Tratamento
14.
Pediatr Cardiol ; 35(2): 323-31, 2014 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-24005901

RESUMO

It is universally recognized that the prevention of sudden cardiac death (SCD) in youth is an important public health initiative. The best approach remains uncertain. Many European and Asian countries support the use of electrocardiograms (ECGs). In the United States, this is highly controversial. Many debate its cost-effectiveness. We designed a comprehensive economic model of two of the most prevalent causes of SCD identifiable by ECG, hypertrophic cardiomyopathy (HCM) and long QT syndrome (LQTS), to determine the drivers of uncertainty in the estimate of cost-effectiveness. We compared the cost-effectiveness of screening with history and physical examination (H&P) plus ECG to the current United States standard, H&P alone, for the detection and treatment of HCM and LQTS. We used a Markov model on a theoretical cohort of healthy 12-year-olds over a 70-year time horizon from a societal perspective, employing extensive univariable and probabilistic sensitivity analyses, to determine drivers of costs and effectiveness. The incremental cost-effectiveness of adding ECGs to H&Ps was $41,400/life-year saved. The model was highly sensitive to the effect of identification and treatment of previously undiagnosed individuals with HCM; however, it was insensitive to many variables commonly assumed to be significant, including the costs of ECGs, echocardiograms, and genetic testing, as well as the sensitivity and specificity of ECGs. No LQTS-related parameters were significant. This study suggests that the key to determining the cost-effectiveness of ECG screening in the United States lies in developing a better understanding of disease progression in the previously undiagnosed HCM population.


Assuntos
Cardiomiopatia Hipertrófica/diagnóstico , Morte Súbita Cardíaca/prevenção & controle , Eletrocardiografia/economia , Síndrome do QT Longo/diagnóstico , Programas de Rastreamento/economia , Modelos Econômicos , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Cardiomiopatia Hipertrófica/complicações , Cardiomiopatia Hipertrófica/fisiopatologia , Criança , Análise Custo-Benefício , Morte Súbita Cardíaca/etiologia , Progressão da Doença , Feminino , Humanos , Incidência , Síndrome do QT Longo/complicações , Síndrome do QT Longo/fisiopatologia , Masculino , Programas de Rastreamento/métodos , Pessoa de Meia-Idade , Fatores de Risco , Estados Unidos/epidemiologia , Adulto Jovem
15.
Cardiol Young ; 24(3): 469-77, 2014 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-23746330

RESUMO

PURPOSE: The aim of this study was to compare associations between generic versus disease-specific functional health status assessments and patient and clinical characteristics for patients with severe congenital heart disease. METHODS: This was a cross-sectional observational study involving 325 single ventricle patients, aged 10-18 years, after Fontan procedure. Enrolled patients underwent a medical history review, laboratory testing, and assessment of the functional health status by completion of the generic Child Report Child Health Questionnaire and the disease-specific Congenital Heart Adolescent and Teenage questionnaire. Correlated conceptually equivalent domains from both questionnaires were identified and their associations with patient and clinical variables were compared. RESULTS: From the generic assessment, patients perceived marginally lower physical functioning (p = 0.05) but greater freedom from bodily pain compared with a normal population (p < 0.001). The equivalent physical functioning/limitations domain of the generic instrument, compared with the disease-specific instrument, had similar associations (higher multi-variable model R²) with medical history variables (R² = 0.14 versus R² = 0.12, respectively) and stronger associations with exercise testing variables (R² = 0.22 versus R² = 0.06). Similarly, the corresponding freedom from bodily pain/symptoms domains from both questionnaires showed a greater association for the generic instrument with medical history variables (R² = 0.15 versus R² = 0.09, respectively) and non-cardiac conditions (R² = 0.13 versus R² = 0.06). The associations of each questionnaire with echocardiographic results, cardiac magnetic resonance imaging results, and serum brain natriuretic peptide levels were uniformly weak (R² range <0.01 to 0.04). CONCLUSIONS: Assessment of the physical functional health status using generic and disease-specific instruments yields few differences with regard to associations between conceptually similar domains and patient and clinical characteristics for adolescents after Fontan procedure.


Assuntos
Técnica de Fontan , Nível de Saúde , Cardiopatias Congênitas/cirurgia , Adolescente , Criança , Estudos Transversais , Feminino , Humanos , Masculino , Inquéritos e Questionários
16.
Heart Rhythm ; 21(4): 454-461, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-37981292

RESUMO

BACKGROUND: Congenital complete heart block (CCHB) is seen in 1:15,000-1:20,000 live births, with risk of left ventricular (LV) dysfunction or dilated cardiomyopathy in 7%-23% of subjects. OBJECTIVE: The purpose of this study was to investigate serial changes in LV size and systolic function in paced CCHB subjects to examine the effect of time from pacemaker on echocardiographic parameters. METHODS: Single-center retrospective cohort analysis of paced CCHB subjects was performed. Echocardiographic data were collected before and after pacemaker placement. Linear mixed effect regression of left ventricular end-diastolic dimension (LVEDD) z-score, left ventricular shortening fraction (LVSF), and left ventricular ejection fraction (LVEF) was performed, with slopes compared before and after pacemaker placement. RESULTS: Of 114 CCHB subjects, 52 had echocardiographic data before and after pacemaker placement. Median age at CCHB diagnosis was 0.6 [interquartile range 0.0-3.5] years; age at pacemaker placement 3.4 [0.5-9.0] years; and pacing duration 10.8 [5.2-13.7] years. Estimated LVEDD z-score was 1.4 at pacemaker placement and decreased -0.08 per year (95% confidence interval [CI] -0.12 to -0.04; P = .002) to 0.2 (95% CI -0.3 to +0.3) 15 years postplacement. Estimated LVSF decreased -1.1% per year (95% CI -1.7% to -0.6%; P <.001) from 6 months prepacemaker placement to 34% (95% CI 32%-37%) 4 years postplacement. There was no significant change in LVSF between 4 and 15 years postplacement. Estimated LVEF did not change significantly after pacemaker placement, with estimated LVEF 59% (95% CI 55%-62%) 15 years postplacement. CONCLUSION: In 52 paced CCHB subjects, estimated LVEDD z-score decreased significantly after pacemaker placement, and estimated LVSF and LVEF remained within normal limits at 15 years postpacemaker placement.


Assuntos
Bloqueio Cardíaco/congênito , Marca-Passo Artificial , Disfunção Ventricular Esquerda , Humanos , Recém-Nascido , Lactente , Pré-Escolar , Criança , Volume Sistólico , Função Ventricular Esquerda , Estudos Retrospectivos , Ecocardiografia , Estimulação Cardíaca Artificial
17.
Curr Opin Cardiol ; 28(2): 139-51, 2013 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-23370230

RESUMO

PURPOSE OF REVIEW: Frequent media reports of sudden cardiac arrest or death (SCA/SCD) keep alive a debate as to how best to prevent these tragedies. Several new studies in the past 2 years serve as an impetus to reframe the debate into a reasonable discussion that seeks to obtain more evidence wherever needed and to develop a consensus wherever possible. RECENT FINDINGS: Since the report from Italy of the 89% decrease in SCD over 25 years of an ECG-based cardiovascular screening program, proponents and opponents of ECG screening have been busily debating. Multiple studies on screening have shown that adding an ECG to a history and physical examination is more sensitive than history and physical examination alone in identifying those potentially at risk. A major gap exists regarding managing these new 'patients' as their clinical course is not known. Reports, without data, have warned of unintended or harmful consequences of ECG screening. Economic models have shown cost-effectiveness of ECG screening to be variable. SUMMARY: Studies suggest that adding an ECG to the screening is a very reasonable effort that will identify at-risk youth and prevent SCD, but more information is needed. If data support the addition of an ECG, efforts should be made to make this opportunity available.


Assuntos
Morte Súbita Cardíaca/prevenção & controle , Programas de Rastreamento/estatística & dados numéricos , Criança , Análise Custo-Benefício , Morte Súbita Cardíaca/epidemiologia , Eletrocardiografia , Humanos , Incidência , Modelos Econômicos , Prevalência , Esportes
18.
Br J Sports Med ; 47(3): 125-36, 2013 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-23303759

RESUMO

Electrocardiographic changes in athletes are common and usually reflect benign structural and electrical remodelling of the heart as a physiological adaptation to regular and sustained physical training (athlete's heart). The ability to identify an abnormality on the 12-lead ECG, suggestive of underlying cardiac disease associated with sudden cardiac death (SCD), is based on a sound working knowledge of the normal ECG characteristics within the athletic population. This document will assist physicians in identifying normal ECG patterns commonly found in athletes. The ECG findings presented as normal in athletes were established by an international consensus panel of experts in sports cardiology and sports medicine.


Assuntos
Adaptação Fisiológica/fisiologia , Arritmias Cardíacas/diagnóstico , Eletrocardiografia , Esportes/fisiologia , Arritmias Cardíacas/fisiopatologia , População Negra , Cardiomegalia Induzida por Exercícios/fisiologia , Diagnóstico Diferencial , Feminino , Humanos , Hipertrofia Ventricular Esquerda/diagnóstico , Hipertrofia Ventricular Esquerda/fisiopatologia , Masculino , Valores de Referência
19.
Br J Sports Med ; 47(3): 122-4, 2013 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-23303758

RESUMO

Sudden cardiac death (SCD) is the leading cause of death in athletes during sport. Whether obtained for screening or diagnostic purposes, an ECG increases the ability to detect underlying cardiovascular conditions that may increase the risk for SCD. In most countries, there is a shortage of physician expertise in the interpretation of an athlete's ECG. A critical need exists for physician education in modern ECG interpretation that distinguishes normal physiological adaptations in athletes from abnormal findings suggestive of pathology. On 13-14 February 2012, an international group of experts in sports cardiology and sports medicine convened in Seattle, Washington, to define contemporary standards for ECG interpretation in athletes. The objective of the meeting was to develop a comprehensive training resource to help physicians distinguish normal ECG alterations in athletes from abnormal ECG findings that require additional evaluation for conditions associated with SCD.


Assuntos
Competência Clínica/normas , Morte Súbita Cardíaca/prevenção & controle , Eletrocardiografia/normas , Medicina Esportiva/normas , Esportes/fisiologia , Diagnóstico Precoce , Educação a Distância , Educação Médica/métodos , Humanos , Internet , Padrões de Referência , Medicina Esportiva/educação
20.
Br J Sports Med ; 47(3): 137-52, 2013 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-23303760

RESUMO

Cardiomyopathies are a heterogeneous group of heart muscle diseases and collectively are the leading cause of sudden cardiac death (SCD) in young athletes. The 12-lead ECG is utilised as both a screening and diagnostic tool for detecting conditions associated with SCD. Fundamental to the appropriate evaluation of athletes undergoing ECG is an understanding of the ECG findings that may indicate the presence of an underlying pathological cardiac disorder. This article describes ECG findings present in cardiomyopathies afflicting young athletes and outlines appropriate steps for further evaluation of these ECG abnormalities. The ECG findings defined as abnormal in athletes were established by an international consensus panel of experts in sports cardiology and sports medicine.


Assuntos
Cardiomiopatias/diagnóstico , Eletrocardiografia , Esportes/fisiologia , Arritmias Cardíacas/diagnóstico , Arritmias Cardíacas/etiologia , População Negra , Cardiomegalia Induzida por Exercícios/fisiologia , Cardiomiopatias/etnologia , Morte Súbita Cardíaca/prevenção & controle , Diagnóstico Diferencial , Ecocardiografia , Humanos
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