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1.
Pediatr Cardiol ; 2023 Sep 09.
Artículo en Inglés | MEDLINE | ID: mdl-37684488

RESUMEN

Hypertrophic cardiomyopathy (HCM), a common cardiomyopathy in children, is an important cause of morbidity and mortality. Early recognition and appropriate management are important. An electrocardiogram (ECG) is often used as a screening tool in children to detect heart disease. The ECG patterns in children with HCM are not well described.ECGs collected from an international cohort of children, and adolescents (≤ 21 years) with HCM were reviewed. 482 ECGs met inclusion criteria. Age ranged from 1 day to 21 years, median 13 years. Of the 482 ECGs, 57 (12%) were normal. The most common abnormalities noted were left ventricular hypertrophy (LVH) in 108/482 (22%) and biventricular hypertrophy (BVH) in 116/482 (24%) Of the patients with LVH/BVH (n = 224), 135 (60%) also had a strain pattern (LVH in 83, BVH in 52). Isolated strain pattern (in the absence of criteria for hypertrophy) was seen in 43/482 (9%). Isolated pathologic Q waves were seen in 71/482 (15%). Pediatric HCM, 88% have an abnormal ECG. The most common ECG abnormalities were LVH or BVH with or without strain. Strain pattern without hypertrophy and a pathologic Q wave were present in a significant proportion (24%) of patients. Thus, a significant number of children with HCM have ECG abnormalities that are not typical for "hypertrophy". The presence of the ECG abnormalities described above in a child should prompt further examination with an echocardiogram to rule out HCM.

2.
Pediatr Cardiol ; 44(5): 1057-1067, 2023 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-36508019

RESUMEN

The evolving breadth and complexity of the contemporary pediatric cardiology specialty requires regular, systematic analysis of the practice to ensure that training and certification requirements address the demands of real-world clinical experience. We report the process of the American Board of Pediatrics (ABP) for conducting such a practice analysis and revising the test content outline (TCO) for the pediatric cardiology subspecialty certification exam. A panel of 15 pediatric cardiologists conducted seven 2-h virtual meetings, during which they identified 37 unique tasks that represent the work a pediatric cardiologist may reasonably expect to perform within the first 5 years after training. These tasks were grouped into nine performance domains, similar to the entrustable professional activities (EPA), previously endorsed by the ABP in collaboration with the pediatric cardiology education community, and which represent the critical activities of the profession. The panel then enumerated the knowledge, skills, and abilities necessary to perform each task. These deliberations resulted in two work products: a practice analysis document (PAD) and subspecialty board TCO based on testable knowledge, skills, and abilities. Survey assessments of the panel's work were then distributed to pediatric cardiology fellowship program directors and to practicing pediatric cardiologists for their input, which largely aligned with the panel's recommendations. Survey responses were considered in the final revisions of the PAD and TCO. This approach to practice analysis proved to be an efficient process for describing the work performed by today's pediatric cardiologists and the knowledge, skills, and abilities needed to competently perform that work.


Asunto(s)
Cardiología , Pediatría , Humanos , Estados Unidos , Niño , Certificación , Competencia Clínica , Curriculum , Cardiología/educación , Pediatría/educación
3.
Clin Sports Med ; 41(3): 485-510, 2022 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-35710274

RESUMEN

Provide a brief summary of your article (100-150 words; no references or figures/tables). The synopsis appears only in the table of contents and is often used by indexing services such as PubMed. Genetic arrhythmia syndromes are rare, yet harbor the potential for highly consequential, often unpredictable arrhythmias or sudden death events. There has been historical uncertainty regarding the correct advice to offer to affected patients who are reasonably wanting to participate in sporting and athletic endeavors. In some cases, this had led to abundantly cautious disqualifications, depriving individuals from participation unnecessarily. Societal guidance and expert opinion has evolved significantly over the last decade or 2, along with our understanding of the genetics and natural history of these conditions, and the emphasis has switched toward shared decision making with respect to the decision to participate or not, with patients and families becoming better informed, and willing participants in the decision making process. This review aims to give a brief update of the salient issues for the busy physician concerning these syndromes and to provide a framework for approaching their management in the otherwise aspirational or keen sports participant.


Asunto(s)
Arritmias Cardíacas , Deportes , Arritmias Cardíacas/genética , Arritmias Cardíacas/terapia , Muerte Súbita Cardíaca/prevención & control , Ejercicio Físico , Humanos , Síndrome
4.
Europace ; 21(11): 1725-1732, 2019 Nov 01.
Artículo en Inglés | MEDLINE | ID: mdl-31408100

RESUMEN

AIMS: Pathogenic gain-of-function variants in CACAN1C cause type-8 long QT syndrome (LQT8). We sought to describe the electrocardiographic features in LQT8 and utilize molecular modelling to gain mechanistic insights into its genetic culprits. METHODS AND RESULTS: Rare variants in CACNA1C were identified from genetic testing laboratories. Treating physicians provided clinical information. Variant pathogenicity was independently assessed according to recent guidelines. Pathogenic (P) and likely pathogenic (LP) variants were mapped onto a 3D modelled structure of the Cav1.2 protein. Nine P/LP variants, identified in 23 patients from 19 families with non-syndromic LQTS were identified. Six variants, found in 79% of families, clustered to a 4-residue section in the cytosolic II-III loop region which forms a region capable of binding STAC SH3 domains. Therefore, variants may affect binding of SH3-domain containing proteins. Arrhythmic events occurred in similar proportions of patients with II-III loop variants and with other P/LP variants (53% vs. 48%, P = 0.41) despite shorter QTc intervals (477 ± 31 ms vs. 515 ± 37 ms, P = 0.03). A history of sudden death was reported only in families with II-III loop variants (60% vs. 0%, P = 0.03). The predominant T-wave morphology was a late peaking T wave with a steep descending limb. Exercise testing demonstrated QTc prolongation on standing and at 4 min recovery after exercise. CONCLUSION: The majority of P/LP variants in patients with CACNA1C-mediated LQT8 cluster in an SH3-binding domain of the cytosolic II-III loop. This represents a 'mutation hotspot' in LQT8. A late-peaking T wave with a steep descending limb and QT prolongation on exercise are commonly seen.


Asunto(s)
Canales de Calcio Tipo L/genética , ADN/genética , Síndrome de QT Prolongado/genética , Mutación Missense , Canales de Calcio Tipo L/metabolismo , Análisis Mutacional de ADN , Electrocardiografía/métodos , Femenino , Estudios de Seguimiento , Pruebas Genéticas/métodos , Humanos , Síndrome de QT Prolongado/metabolismo , Síndrome de QT Prolongado/fisiopatología , Masculino , Linaje , Fenotipo , Unión Proteica , Estudios Retrospectivos
5.
Pediatr Cardiol ; 40(6): 1253-1257, 2019 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-31263917

RESUMEN

Obesity is associated with additional left ventricular hypertrophy (LVH) in adults with hypertrophic cardiomyopathy (HCM). It is not known whether obesity can lead to further LVH in children with HCM. Echocardiographic LV dimensions were determined in 504 children with HCM. Measurements of interventricular septal thickness (IVST) and posterior wall thickness (PWT), and patients' weight and height were recorded. Obesity was defined as a body mass index (BMI) ≥ 99th percentile for age and sex. IVST data was available for 498 and PWT data for 484 patients. Patient age ranged from 2 to 20 years (mean ± SD, 12.5 ± 3.9) and 340 (68%) were males. Overall, patient BMI ranged from 7 to 50 (22.7 ± 6.1). Obesity (BMI 18-50, mean 29.1) was present in 140 children aged 2-19.6 (11.3 ± 4.1). The overall mean IVST was 20.5 ± 9.6 mm and the overall mean PWT was 11.0 ± 8.4 mm. The mean IVST in the obese patients was 21.6 ± 10.0 mm and mean PWT was 13.3 ± 14.7 mm. The mean IVST in the non-obese patients was 20.1 ± 9.5 mm and mean PWT was 10.4 ± 4.3 mm. Obesity was not significantly associated with IVST (p = 0.12), but was associated with increased PWT (0.0011). Obesity is associated with increased PWT but not IVST in children with HCM. Whether obesity and its impact on LVH influences clinical outcomes in children with HCM needs to be studied.


Asunto(s)
Cardiomiopatía Hipertrófica/complicaciones , Ventrículos Cardíacos/patología , Obesidad/complicaciones , Tabique Interventricular/patología , Adolescente , Índice de Masa Corporal , Cardiomiopatía Hipertrófica/fisiopatología , Niño , Preescolar , Ecocardiografía , Femenino , Humanos , Masculino , Adulto Joven
6.
Heart Rhythm ; 16(10): 1462-1467, 2019 10.
Artículo en Inglés | MEDLINE | ID: mdl-31026510

RESUMEN

BACKGROUND: Predictors of risk of lethal arrhythmic events (LAE) is poorly understood and may differ from adults in children with hypertrophic cardiomyopathy (HCM). OBJECTIVE: The purpose of this study was to determine predictors of LAE in children with HCM. METHODS: A retrospective data collection was performed on 446 children and teenagers 20 years and younger (290 [65%] male; mean age 10.1 ± 5.7 years) with idiopathic HCM from 35 centers. Patients were classified as group 1 (HCM with LAE) if having a secondary prevention implantable cardioverter-defibrillator (ICD) or primary prevention ICD with appropriate interventions or group 2 (HCM without LAE) if having a primary prevention ICD without appropriate interventions. RESULTS: There were 152 children (34%) in group 1 and 294 (66%) in group 2. Risk factors for group 1 by univariate analysis were septal thickness, posterior left ventricular (LV) wall thickness, lower LV outflow gradient, and Q wave > 3 mm in inferior electrocardiographic leads. Factors not associated with LAE were family history of SCD, abnormal blood pressure response to exercise, and ventricular tachycardia on ambulatory electrocardiographic monitoring. Risk factors for SCD by multivariate analysis were age at ICD placement (hazard ratio [HR] 0.9; P = .0025), LV posterior wall thickness z score (HR 1.02; P < .005), and LV outflow gradient < 30 mm Hg (HR 2.0; P < .006). LV posterior wall thickness z score ≥ 5 was associated with LAE. CONCLUSION: Risk factors for LAE appear different in children compared to adults. Conventional adult risk factors were not significant in children. Further prospective studies are needed to improve risk stratification for LAE in children with HCM.


Asunto(s)
Arritmias Cardíacas/terapia , Cardiomiopatía Hipertrófica/complicaciones , Muerte Súbita Cardíaca/prevención & control , Desfibriladores Implantables , Adolescente , Arritmias Cardíacas/diagnóstico por imagen , Arritmias Cardíacas/etiología , Cardiomiopatía Hipertrófica/diagnóstico , Niño , Preescolar , Estudios de Cohortes , Ecocardiografía/métodos , Electrocardiografía/métodos , Femenino , Estudios de Seguimiento , Hospitales Pediátricos , Humanos , Internacionalidad , Estimación de Kaplan-Meier , Masculino , Modelos de Riesgos Proporcionales , Estudios Retrospectivos , Medición de Riesgo , Índice de Severidad de la Enfermedad , Resultado del Tratamiento , Adulto Joven
7.
Circulation ; 136(19): e273-e344, 2017 Nov 07.
Artículo en Inglés | MEDLINE | ID: mdl-28974521

RESUMEN

BACKGROUND AND PURPOSE: This scientific statement provides an interprofessional, comprehensive review of evidence and recommendations for indications, duration, and implementation of continuous electro cardiographic monitoring of hospitalized patients. Since the original practice standards were published in 2004, new issues have emerged that need to be addressed: overuse of arrhythmia monitoring among a variety of patient populations, appropriate use of ischemia and QT-interval monitoring among select populations, alarm management, and documentation in electronic health records. METHODS: Authors were commissioned by the American Heart Association and included experts from general cardiology, electrophysiology (adult and pediatric), and interventional cardiology, as well as a hospitalist and experts in alarm management. Strict adherence to the American Heart Association conflict of interest policy was maintained throughout the consensus process. Authors were assigned topics relevant to their areas of expertise, reviewed the literature with an emphasis on publications since the prior practice standards, and drafted recommendations on indications and duration for electrocardiographic monitoring in accordance with the American Heart Association Level of Evidence grading algorithm that was in place at the time of commissioning. RESULTS: The comprehensive document is grouped into 5 sections: (1) Overview of Arrhythmia, Ischemia, and QTc Monitoring; (2) Recommendations for Indication and Duration of Electrocardiographic Monitoring presented by patient population; (3) Organizational Aspects: Alarm Management, Education of Staff, and Documentation; (4) Implementation of Practice Standards; and (5) Call for Research. CONCLUSIONS: Many of the recommendations are based on limited data, so authors conclude with specific questions for further research.


Asunto(s)
American Heart Association , Arritmias Cardíacas/diagnóstico , Servicio de Cardiología en Hospital/normas , Electrocardiografía/normas , Hospitalización , Arritmias Cardíacas/fisiopatología , Arritmias Cardíacas/terapia , Alarmas Clínicas/normas , Consenso , Documentación/normas , Electrocardiografía Ambulatoria/normas , Registros Electrónicos de Salud/normas , Medicina Basada en la Evidencia/normas , Prueba de Esfuerzo/normas , Control de Formularios y Registros/normas , Humanos , Valor Predictivo de las Pruebas , Pronóstico , Estados Unidos
8.
Circulation ; 126(17): 2146-72, 2012 Oct 23.
Artículo en Inglés | MEDLINE | ID: mdl-23008437

RESUMEN

Significant mortality benefits have been documented in recipients of implantable cardioverter defibrillators (ICDs); however, the psychosocial distress created by the underlying arrhythmia and its potential treatments in patients and family members may be underappreciated by clinical care teams. The disentanglement of cardiac disease and device-related concerns is difficult. The majority of ICD patients and families successfully adjust to the ICD, but optimal care pathways may require additional psychosocial attention to all ICD patients and particularly those experiencing psychosocial distress. This state-of-the-science report was developed on the basis of an analysis and critique of existing science to (1) describe the psychological and quality-of-life outcomes after receipt of an ICD and describe related factors, such as patient characteristics; (2) describe the concerns and educational/informational needs of ICD patients and their family members; (3) outline the evidence that supports interventions for improving educational and psychological outcomes for ICD patients; (4) provide recommendations for clinical approaches for improving patient outcomes; and (5) identify priorities for future research in this area. The ultimate goal of this statement is to improve the precision of identification and care of psychosocial distress in ICD patients to maximize the derived benefit of the ICD.


Asunto(s)
American Heart Association , Desfibriladores Implantables/psicología , Familia/psicología , Educación del Paciente como Asunto/métodos , Guías de Práctica Clínica como Asunto , Humanos , Guías de Práctica Clínica como Asunto/normas , Resultado del Tratamiento , Estados Unidos
9.
Europace ; 13(4): 548-54, 2011 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-21296778

RESUMEN

INTRODUCTION: We sought to determine the incidence, predictors, and consequences of new-onset atrial fibrillation (AF) following epicardial ventricular tachycardia (VT) ablation. METHODS AND RESULTS: A total of 41 patients with no prior history of AF underwent epicardial VT ablation via a percutaneous subxiphoid approach. All patients were monitored continuously for 3 days following ablation and then via implantable cardiac defibrillator (ICD) or Holter monitoring. Mean age was 70.0 ± 11.3 years and mean ejection fraction was 30.3 ± 16.6%. In seven (17%) patients, the right ventricle (RV) was punctured during access with subsequent needle withdrawal without requiring surgical repair. Thirty patients (73%) were treated with amiodarone following ablation. Post-ablation, eight (19.5%) patients had documented new-onset AF within 7 days. All AF patients had clinical symptoms of pericarditis. One patient with AF was maintained on amiodarone post-procedure. Complications of AF included three patients who received inappropriate ICD shocks and one patient who developed a large, left atrial appendage clot. Acutely, all patients responded to short-term medical therapy or electrical cardioversion. At 18.0 ± 9.0 months of follow-up, no patient had recurrence of AF, and all were off antiarrhythmic drugs. One patient had typical atrial flutter requiring catheter ablation. Risk factors for AF included lack of amiodarone immediately after ablation (12.5 vs. 87.9%, P < 0.001), RV puncture (50.0 vs. 9.1%, P = 0.02), and epicardial ablation time >10 min (62.5 vs. 3.0%, P < 0.001). CONCLUSIONS: Atrial fibrillation after epicardial ablation is common and can lead to ICD shocks and atrial thrombus formation. Short-term antiarrhythmic drug therapy and ICD reprogramming should be considered after epicardial VT ablation.


Asunto(s)
Fibrilación Atrial/epidemiología , Ablación por Catéter/efectos adversos , Taquicardia Ventricular/cirugía , Adulto , Anciano , Fibrilación Atrial/fisiopatología , Fibrilación Atrial/terapia , Desfibriladores Implantables , Electrocardiografía Ambulatoria , Femenino , Estudios de Seguimiento , Humanos , Incidencia , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Factores de Riesgo , Resultado del Tratamiento
10.
Heart Rhythm ; 7(5): 604-9, 2010 May.
Artículo en Inglés | MEDLINE | ID: mdl-20156612

RESUMEN

BACKGROUND: Nonsurgical subxiphoid pericardial access may be useful in ventricular tachycardia ablation and other electrophysiologic procedures but has a risk of right ventricular puncture. OBJECTIVE: The purpose of this study was to identify a signature pressure frequency that would help identify the pericardial space and guide access. METHODS: The study consisted of 20 patients (8 women and 12 men; mean age 59.1 +/- 14.2 years; left ventricular ejection fraction 25.2% +/- 12.2%; failed 1.8 +/- 0.5 endocardial ablations; unresponsive to 2.0 +/- 1.0 antiarrhythmic drugs; 6 ischemic cardiomyopathy, 12 nonischemic cardiomyopathy, 2 normal heart; 4 previous sternotomy) undergoing epicardial ventricular tachycardia ablation. After pericardial access was obtained, a 10Fr long sheath was used to record pressure inside the pericardium and pleural space. Pressures were analyzed using a fast Fourier transform to identify dominant frequencies in each chamber. RESULTS: Mean pressures in the pleural space and the pericardium were not different (7.7 +/- 1.9 mmHg vs 7.8 +/- 0.9 mmHg, respectively). However, the pericardial space in each patient demonstrated two frequency peaks that correlated with heart rate (1.16 +/- 0.21 Hz) and respiratory rate (0.20 +/- 0.01 Hz), whereas the pleural space in each patient had a single peak correlating with respiratory rate (0.20 +/- 0.01 Hz). CONCLUSION: The pericardial space demonstrates a signature pressure frequency that is significantly different from the surrounding space. This difference may make minimally invasive subxiphoid pericardial access safer for nonsurgeons and may have important implications for electrophysiologic procedures.


Asunto(s)
Ablación por Catéter/métodos , Pericardio , Presión , Taquicardia Ventricular/cirugía , Tórax , Ablación por Catéter/efectos adversos , Electrofisiología , Femenino , Análisis de Fourier , Ventrículos Cardíacos/lesiones , Humanos , Masculino , Persona de Mediana Edad , Estudios Prospectivos , Factores de Riesgo , Estadística como Asunto , Esternotomía , Volumen Sistólico , Factores de Tiempo , Función Ventricular Izquierda
11.
J Cardiovasc Electrophysiol ; 21(6): 678-84, 2010 Jun 01.
Artículo en Inglés | MEDLINE | ID: mdl-20102427

RESUMEN

BACKGROUND: Ablation of ventricular tachycardia (VT) reduces implantable cardioverter defibrillator shocks. Intracardiac ultrasound (ICE) can visualize and quantify the function of all left ventricular wall segments. We thus hypothesized that ICE could identify scar tissue and provide a guide to facilitate substrate-guided VT ablation. METHODS: Eighteen patients underwent VT ablation with real time ICE mapping from the right atrium and ventricle with online 3D-image reconstruction of scar segments. The left ventricle was also scar mapped by traditional electroanatomic mapping (CARTO) for comparison. Images from these 2 scar mapping techniques were compared to each other as well as to a preprocedure transthoracic echocardiogram. RESULTS: The average age was 65 +/- 12 years and 12 (67%) were male (15 [83%] had ischemic cardiomyopathy). Two patients (12%) had recurrence of their clinical VT (1 remained on an antiarrhythmic medication, the other had a repeat ablation) over a follow-up of 127 +/- 33 days. No periprocedural or long-term adverse events occurred. A total of 248 wall segments were analyzed. All 3 modalities were concordant in scar identification in 193 (78%) segments. The ICE segments correlated with the electroanatomic map in 213 (86%) segments versus 198 (80%), which correlated with transthoracic echocardiography and electroanatomic mapping (P = 0.046). Specifically, the ICE wall motion scores were closer to the electroanatomic mapping in the basal segments and showed a higher accuracy in ischemic heart disease. CONCLUSION: These data demonstrate that real time ICE images provide accurate chamber geometries and scar boundaries of the left ventricle. These scar borders were more accurate than transthoracic echocardiography and illustrate the feasibility of ICE for substrate-based ablation for VT.


Asunto(s)
Ablación por Catéter/métodos , Procesamiento de Imagen Asistido por Computador/métodos , Taquicardia Ventricular/diagnóstico por imagen , Taquicardia Ventricular/cirugía , Anciano , Ecocardiografía , Electrocardiografía , Técnicas Electrofisiológicas Cardíacas/métodos , Femenino , Fluoroscopía , Humanos , Masculino , Persona de Mediana Edad , Cirugía Asistida por Computador
12.
Circ Arrhythm Electrophysiol ; 2(6): 611-9, 2009 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-20009075

RESUMEN

BACKGROUND: Catheter ablation of atrial fibrillation is currently guided by x-ray fluoroscopy. The associated radiation risk to patients and medical staff may be significant. We report an atrial fibrillation ablation technique using intracardiac echocardiography (ICE) and electroanatomic mapping without fluoroscopy. METHODS AND RESULTS: Twenty-one patients with atrial fibrillation (age, 42 to 73 years; 14 male; 14 paroxysmal, 7 persistent; body mass index, 26 to 38) underwent ablation. A decapolar catheter was advanced through the left subclavian vein until stable coronary sinus electrograms appeared on all electrodes. Two 9F sheaths were advanced transfemorally over a guide wire to the right atrium. A rotational ICE catheter was advanced through a deflectable sheath. Double transseptal puncture was performed with ICE guidance and facilitated by electrocautery. A 3D MRI left atrial image was registered to the ostia of the pulmonary veins using ICE. Catheter ablation was performed using ICE and electroanatomic mapping navigation. In 19 cases, no fluoroscopy was used and the staff did not wear protective lead. In 2 cases, 2 to 16 minutes of fluoroscopy was used to assist transseptal puncture. Median procedure time was 208 (188 to 221) minutes; coronary sinus cannulation took 5 (2 to 26) minutes; double transseptal took 26 (17 to 40) minutes; left atrial ablation time was 103 (90 to 127) minutes. All patients underwent circumferential pulmonary vein ablation and 8 patients underwent additional left atrial ablation. There were no procedure-related complications. CONCLUSIONS: Catheter ablation of atrial fibrillation without fluoroscopy is feasible and merits further attention. This technique may be especially helpful in preventing x-ray exposure in children, pregnant women, and obese patients undergoing left atrial ablation.


Asunto(s)
Fibrilación Atrial/cirugía , Ablación por Catéter , Ecocardiografía , Técnicas Electrofisiológicas Cardíacas , Ultrasonografía Intervencional , Adulto , Anciano , Fibrilación Atrial/diagnóstico , Fibrilación Atrial/diagnóstico por imagen , Femenino , Fluoroscopía/efectos adversos , Humanos , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Valor Predictivo de las Pruebas , Estudios Prospectivos , Radiografía Intervencional/efectos adversos , Resultado del Tratamiento
13.
Congenit Heart Dis ; 4(5): 356-61, 2009.
Artículo en Inglés | MEDLINE | ID: mdl-19740190

RESUMEN

OBJECTIVE: We studied, as a physiological benchmark, acute effects of right ventricular (RV) apical, RV outflow, and left ventricular (LV) pacing in children with normal cardiac function on LV and RV function and ventricular-ventricular interactions. DESIGN: The design of the study was a prospective, acute intervention. SETTING: The study was conducted in a tertiary care electrophysiology laboratory. Population and Methods. Seven children (mean +/- SD, 12 +/- 4 years) were paced after accessory pathway ablation, at baseline (AOO), and with atrioventricular pacing (DOO) from the RV apex, RV outflow, and left ventricle. OUTCOME MEASURES: Right ventricular dP/dT(max) and RV dP/dT(neg) (high-fidelity transducer-tipped catheters, Millar Instruments, Houston, TX, USA), cardiac index (Fick), blood pressure, and QRS duration were measured at each pacing condition. Intra- and interventricular mechanical dyssynchrony, systolic- and diastolic peak tissue velocities, and isovolumic acceleration were recorded by tissue Doppler imaging at the lateral mitral, septal, and tricuspid annuli at each condition. Results at each pacing condition were compared by repeated-measures analysis of variance. Results. Pacing prolonged QRS duration, causing electrical dyssynchrony (86 +/- 19 ms [baseline], 141 +/- 44 ms [RV apex], 121 +/- 18 ms [RV outflow], and 136 +/- 34 ms [LV], P < .01). Right ventricular outflow pacing caused LV intraventricular delay (63 +/- 52 vs. 12 +/- 7 ms, P < .05). Right ventricular apical pacing caused interventricular delay (61 +/- 29 vs. 25 +/- 18 ms, P < .05). There were no significant changes in blood pressure, cardiac index, RV dp/dT(max), RV dP/dT(neg), regional tissue velocities, or isovolumic acceleration during any of the pacing conditions, indicating preserved ventricular function and hemodynamics. No important ventricular-ventricular interactions were seen. CONCLUSIONS: In children with normal cardiac anatomy and function, single-site RV apical, RV outflow, and LV pacing induce electromechanical dyssynchrony without significantly changing ventricular function or hemodynamics, or adversely affecting ventricular-ventricular interactions.


Asunto(s)
Estimulación Cardíaca Artificial/métodos , Hemodinámica , Taquicardia Supraventricular/fisiopatología , Función Ventricular Izquierda , Función Ventricular Derecha , Adolescente , Presión Sanguínea , Ablación por Catéter , Niño , Criocirugía , Ecocardiografía Doppler de Pulso , Técnicas Electrofisiológicas Cardíacas , Femenino , Humanos , Masculino , Contracción Miocárdica , Estudios Prospectivos , Taquicardia Supraventricular/diagnóstico por imagen , Taquicardia Supraventricular/cirugía
14.
Curr Opin Pediatr ; 21(5): 573-8, 2009 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-19617828

RESUMEN

PURPOSE OF REVIEW: Advances in understanding the biophysical underpinnings of long QT syndrome have provided growing insight into the risk of this syndrome in the pediatric population. This review focuses on developments in this area as reflected in the recent literature. RECENT FINDINGS: QT interval prolongation on the surface ECG is the hallmark of long QT syndrome. This prolongation reflects protracted ventricular repolarization, primarily due to mutations in genes coding for cardiac ion channels. To date, 12 different genes have been implicated, and current genetic testing methods can provide a specific diagnosis in approximately 70% of patients. Clinical indicators, including age, sex, corrected QT duration, and prior syncope are the most powerful predictors of future life-threatening cardiac events. However, diagnosis, risk assessment, and therapeutic strategies are being guided by genetic analysis to an increasing degree. SUMMARY: Impressive advancements have been made in understanding the genetic and clinical determinants of this heterogeneous syndrome. As genetic testing techniques become more robust, the ability to assess risk in affected individuals and tailor therapy will improve.


Asunto(s)
Síndrome de QT Prolongado/diagnóstico , Síndrome de QT Prolongado/etiología , Adolescente , Factores de Edad , Niño , Preescolar , Electrocardiografía , Genotipo , Humanos , Lactante , Síndrome de QT Prolongado/terapia , Fenotipo , Factores de Riesgo , Factores Sexuales , Adulto Joven
15.
J Cardiovasc Electrophysiol ; 20(5): 539-44, 2009 May.
Artículo en Inglés | MEDLINE | ID: mdl-19017336

RESUMEN

BACKGROUND: Right ventricular (RV) pacing may be detrimental to ventricular function. However, the acute effects of electromechanical dyssynchrony on RV function are not well characterized in children. We studied acute effects of electromechanical dyssynchrony, induced by RV apical and RV outflow pacing, in children with normal hearts, evaluating electromechanical synchrony, hemodynamic response, and RV function. METHODS: Seventeen children (mean +/- SD, 12 +/- 4 years) with normal cardiac structure/function were paced after accessory pathway ablation, at baseline (AOO), and with AV pacing (DOO) from the RV apex and RV outflow. QRS duration was determined from surface ECG. Intra- and interventricular mechanical dyssynchrony and regional ventricular function were determined using tissue Doppler imaging. Global RV systolic and diastolic functions were assessed by RV dP/dT(max) and RV dP/dT(neg) using pressure-tipped transducers. Regional RV function was assessed by tissue Doppler imaging. Cardiac index (CI) and blood pressures were measured. RESULTS: RV apical and outflow pacing induced significant electromechanical dyssynchrony manifested by lengthening of the QRS duration, increased LV intraventricular delay (49 +/- 34 ms, 53 +/- 43 ms, respectively, P < 0.001), and increased interventricular delay (60 +/- 29 ms, 55 +/- 37 ms, P < 0.0001) versus AOO pacing. However, there was no change in blood pressure, CI, RV dp/dT(max), RV dP/dT(neg), or regional tissue Doppler velocities, indicating preserved hemodynamics and preserved global and regional RV systolic and diastolic function. CONCLUSIONS: In children with normal cardiac function and structure, pacing-induced electromechanical dyssynchrony did not acutely affect RV systolic and diastolic function and did not acutely alter global hemodynamics. Therefore, electromechanical dyssynchrony may only be an important therapeutic target in the setting of decreased RV function.


Asunto(s)
Estimulación Cardíaca Artificial/métodos , Circulación Coronaria , Contracción Miocárdica , Disfunción Ventricular Izquierda/fisiopatología , Adolescente , Preescolar , Femenino , Humanos , Masculino , Valores de Referencia , Adulto Joven
16.
Heart Rhythm ; 3(5): 557-63, 2006 May.
Artículo en Inglés | MEDLINE | ID: mdl-16648061

RESUMEN

BACKGROUND: Cryoablation for treatment of atrioventricular nodal reentrant tachycardia (AVNRT) is safe and efficacious. Information on the effects of cryoablation on atrioventricular (AV) nodal conduction is limited. OBJECTIVES: The purpose of this study was to evaluate the effects of cryoablation on AV nodal conduction in pediatric patients with AVNRT. METHODS: We retrospectively analyzed electrophysiologic studies before and after successful cryoablation. Patients were divided into two groups: group 1 (n = 22, age 14 +/- 3 years) had baseline discontinuous atrial-to-His interval (AH) conduction curves; and group 2 (n = 13, age 12 +/- 4 years, P = .054) had continuous curves. RESULTS: At baseline, group 1 had longer measurements of maximal AH with A1A2, AV nodal effective refractory period, and AV block cycle length. Postcryoablation, both group 1 and group 2 showed decreases in maximal AH with A1A2 pacing or atrial overdrive pacing and in the finding of PR > or = RR with atrial overdrive pacing (group 1: 55% vs 5%, P < .001; group 2: 69% vs 0%, P < .001). A significant increase in overall AV effective refractory period and a decrease in AV block cycle length were found in group 1 but not group 2. Fifty percent of group 1 patients had complete abolition of slow pathway conduction. CONCLUSION: Successful cryoablation for treatment of AVNRT is associated with a reduction in PR > or = RR and with decreases in maximal AH with A1A2 pacing or atrial overdrive pacing. Further study is needed to determine the usefulness of these parameters for assessment of ablation efficacy or as proxies for AVNRT inducibility.


Asunto(s)
Criocirugía , Técnicas Electrofisiológicas Cardíacas , Taquicardia por Reentrada en el Nodo Atrioventricular/fisiopatología , Taquicardia por Reentrada en el Nodo Atrioventricular/cirugía , Adolescente , Función Atrial , Fascículo Atrioventricular/fisiopatología , Fascículo Atrioventricular/cirugía , Niño , Femenino , Estudios de Seguimiento , Bloqueo Cardíaco/fisiopatología , Bloqueo Cardíaco/cirugía , Humanos , Masculino , Estudios Retrospectivos , Resultado del Tratamiento
17.
Heart Rhythm ; 3(1): 95-101, 2006 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-16399063

RESUMEN

Adult and pediatric interventional electrophysiology practices have been diverging for the past 10 years, and so we review current pediatric ablation practice. Radiofrequency ablation (RFA) is safe and efficacious as documented by recent prospective, multi-center pediatric studies. Computer assisted mapping systems used for complex arrhythmias in adult patients have been successfully deployed in selected pediatric substrates. With increased computational power, decreased catheter size, and increased maneuverability, we expect increased use in the pediatric population. Finally, cryoablation has demonstrated efficacy and safety in locations traditionally associated with increased risk when using RFA, particularly around the AV node. As larger, multi-institutional studies are undertaken, the benefits of this technology in pediatric patients will be better defined.


Asunto(s)
Ablación por Catéter , Adolescente , Niño , Criocirugía , Técnicas Electrofisiológicas Cardíacas , Humanos , Seguridad , Taquicardia/cirugía
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