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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.
J Cardiovasc Electrophysiol ; 33(1): 128-133, 2022 01.
Artículo en Inglés | MEDLINE | ID: mdl-34716972

RESUMEN

This series describes an innovative technique for pacing in patients with sinus node dysfunction after extracardiac Fontan surgery. This transpulmonary approach to the left atrial epi-myocardium has been successfully applied to three patients at two centers and resulted in excellent acute and midterm pacing characteristics without known complications. The principal advantage of this procedure in comparison to prior iterations is the absence of pacing material within the pulmonary venous atrium, so that future systemic thromboembolism risk is minimized. The transpulmonary approach for permanent atrial pacing offers a novel solution to the unique challenges for patients after extracardiac Fontan operation.


Asunto(s)
Procedimiento de Fontan , Cardiopatías Congénitas , Marcapaso Artificial , Estimulación Cardíaca Artificial/métodos , Estudios de Factibilidad , Procedimiento de Fontan/efectos adversos , Cardiopatías Congénitas/cirugía , Humanos , Síndrome del Seno Enfermo/terapia
3.
Cardiol Young ; 32(11): 1820-1825, 2022 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-34986912

RESUMEN

BACKGROUND: Obesity increases the risk of post-operative arrhythmias in adults undergoing cardiac surgery, but little is known regarding the impact of obesity on post-operative arrhythmias after CHD surgery. METHODS: Patients undergoing CHD surgery from 2007 to 2019 were prospectively enrolled in the parent study. Telemetry was assessed daily, with documentation of all arrhythmias. Patients aged 2-20 years were categorised by body mass index percentile for age and sex (underweight <5, normal 5-85, overweight 85-95, and obese >95). Patients aged >20 years were categorised using absolute body mass index. We investigated the impact of body mass index category on arrhythmias using univariate and multivariate analysis. RESULTS: There were 1250 operative cases: 12% underweight, 65% normal weight, 12% overweight, and 11% obese. Post-operative arrhythmias were observed in 38%. Body mass index was significantly higher in those with arrhythmias (18.8 versus 17.8, p = 0.003). There was a linear relationship between body mass index category and incidence of arrhythmias: underweight 33%, normal 38%, overweight 42%, and obese 45% (p = 0.017 for trend). In multivariate analysis, body mass index category was independently associated with post-operative arrhythmias (p = 0.021), with odds ratio 1.64 in obese patients as compared to normal-weight patients (p = 0.036). In addition, aortic cross-clamp time (OR 1.007, p = 0.002) and maximal vasoactive-inotropic score in the first 48 hours (OR 1.03, p = 0.04) were associated with post-operative arrhythmias. CONCLUSION: Body mass index is independently associated with incidence of post-operative arrhythmias in children after CHD surgery.


Asunto(s)
Cardiopatías Congénitas , Delgadez , Niño , Humanos , Adulto Joven , Delgadez/complicaciones , Delgadez/cirugía , Sobrepeso/complicaciones , Factores de Riesgo , Obesidad/complicaciones , Obesidad/epidemiología , Índice de Masa Corporal , Arritmias Cardíacas/etiología , Arritmias Cardíacas/complicaciones , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/cirugía , Estudios Retrospectivos
4.
J Cardiovasc Magn Reson ; 23(1): 108, 2021 10 11.
Artículo en Inglés | MEDLINE | ID: mdl-34629101

RESUMEN

The Society for Cardiovascular Magnetic Resonance (SCMR) is an international society focused on the research, education, and clinical application of cardiovascular magnetic resonance (CMR). Case of the week is a case series hosted on the SCMR website ( https://www.scmr.org ) that demonstrates the utility and importance of CMR in the clinical diagnosis and management of cardiovascular disease. Each case consists of the clinical presentation and a discussion of the condition and the role of CMR in diagnosis and guiding clinical management. The cases are all instructive and helpful in the approach to patient management. We present a digital archive of the 2020 Case of the Week series of 11 cases as a means of further enhancing the education of those interested in CMR and as a means of more readily identifying these cases using a PubMed or similar search engine.


Asunto(s)
Enfermedades Cardiovasculares , Imagen por Resonancia Magnética , Enfermedades Cardiovasculares/diagnóstico por imagen , Enfermedades Cardiovasculares/terapia , Humanos , Espectroscopía de Resonancia Magnética , Valor Predictivo de las Pruebas
5.
J Cardiovasc Electrophysiol ; 31(8): 2043-2048, 2020 08.
Artículo en Inglés | MEDLINE | ID: mdl-32542917

RESUMEN

INTRODUCTION: Experience with catheter ablation of orthodromic reciprocating tachycardia (ORT) and atrioventricular nodal reentrant tachycardia (AVNRT) in young children with hypoplastic left heart syndrome (HLHS) is limited. We report the feasibility, safety, and outcomes of catheter ablation of ORT and AVNRT in children with HLHS. METHODS AND RESULTS: This was a retrospective review of patients with HLHS who underwent catheter ablation for reentrant supraventricular tachycardias (excluding atrial tachycardias) between 2005 and 2017 at a single center. Descriptive data including demographics, clinical history, procedural data, and outcomes were recorded. Ten children with HLHS underwent eleven catheter ablation procedures. Median age and weight at ablation were 2.7 years (range: 0.1-10.5) and 11.4 kg (range: 3.6-30.4), respectively. Tachycardia mechanism was AVNRT in four, ORT in five (two with preexcitation), and both in one. Acute procedural success was 100% and there was no spontaneous recurrence of tachycardia orpreexcitationin median 92 months (range: 21-175 months) follow-up. Five patients underwent subsequent EP studies at catheterization (intracardiac) or after surgery (via epicardial wires): three were noninducible, one after AVNRT ablation had inducible atrial tachycardia, and one after initial ORT ablation had inducible ORT at fenestration closure and underwent successful repeat ablation. Thus, long-term freedom from clinical tachycardia was 100% and from inducible AVNRT or ORT was 80%. CONCLUSION: Transcatheter ablation for ORT and AVNRT in children with HLHS can be performed with excellent acute and long-term success without major complications.


Asunto(s)
Ablación por Catéter , Síndrome del Corazón Izquierdo Hipoplásico , Taquicardia por Reentrada en el Nodo Atrioventricular , Taquicardia Reciprocante , Ablación por Catéter/efectos adversos , Niño , Preescolar , Electrocardiografía , Humanos , Estudios Retrospectivos , Taquicardia por Reentrada en el Nodo Atrioventricular/diagnóstico , Taquicardia por Reentrada en el Nodo Atrioventricular/cirugía , Taquicardia Reciprocante/diagnóstico , Taquicardia Reciprocante/cirugía
6.
J Cardiovasc Electrophysiol ; 30(11): 2599-2602, 2019 11.
Artículo en Inglés | MEDLINE | ID: mdl-31432598

RESUMEN

INTRODUCTION: Current resuscitation guidelines state that the safety of automated external defibrillators (AEDs) in infants less than 1 year of age is unknown. METHODS AND RESULTS: We report successful AED use in a 31-day-old previously healthy infant with out-of-hospital cardiac arrest. Chest compressions began immediately, pediatric AED pads were applied in less than 5 minutes and the initial rhythm was ventricular fibrillation. After two 50 J shocks, return of spontaneous circulation was achieved. She was diagnosed with a rare but previously described syndrome of infant ventricular fibrillation and was discharged to home in good condition after epicardial defibrillator placement. CONCLUSIONS: This case represents, to our knowledge, the youngest patient successfully defibrillated by an AED in a nonmedical setting. Although she received two shocks more than 11 J/kg each, she had no apparent myocardial damage at presentation.


Asunto(s)
Reanimación Cardiopulmonar/métodos , Desfibriladores , Paro Cardíaco Extrahospitalario/terapia , Fibrilación Ventricular/terapia , Reanimación Cardiopulmonar/instrumentación , Femenino , Humanos , Lactante , Paro Cardíaco Extrahospitalario/diagnóstico , Paro Cardíaco Extrahospitalario/fisiopatología , Fibrilación Ventricular/diagnóstico , Fibrilación Ventricular/fisiopatología
7.
Pacing Clin Electrophysiol ; 42(1): 46-57, 2019 01.
Artículo en Inglés | MEDLINE | ID: mdl-30334588

RESUMEN

BACKGROUND: Axillary implant location is an alternative implant location in patients for cardiac implantable electronic devices (CIEDs) for the purposes of improved cosmetic outcome. The impact from the patient's perspective is unknown. The purpose of this study was to compare scar perception scores and quality of life (QOL) in pediatric patients with axillary CIED implant location versus the standard infraclavicular approach. METHODS: This is a multicenter prospective study conducted at eight pediatric centers and it includes patients aged from 8 to 18 years with a CIED. Patients with prior sternotomy were excluded. Scar perception and QOL outcomes were compared between the infraclavicular and axillary implant locations. RESULTS: A total of 141 patients (83 implantable cardioverter defibrillator [ICD]/58 pacemakers) were included, 55 with an axillary device and 86 with an infraclavicular device. Patients with an ICD in the axillary position had better perception of scar appearance and consciousness. Patients in the axillary group reported, on average, a total Pediatric QOL Inventory score that was 6 (1, 11) units higher than the infraclavicular group, after adjusting for sex and race (P = 0.02). CONCLUSIONS: QOL is significantly improved in axillary in comparison to the infraclavicular CIED position, regardless of device type. Scar perception is improved in patients with ICD in the axillary position.


Asunto(s)
Cicatriz/psicología , Desfibriladores Implantables , Estética , Cardiopatías Congénitas/terapia , Marcapaso Artificial , Calidad de Vida , Adolescente , Niño , Estudios Transversales , Femenino , Humanos , Masculino , Estudios Prospectivos , Encuestas y Cuestionarios
8.
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
10.
J Electrocardiol ; 49(1): 42-5, 2016.
Artículo en Inglés | MEDLINE | ID: mdl-26482959

RESUMEN

There is limited longitudinal data on accessory pathway (AP) antegrade conduction throughout childhood, with implications for risk stratification. Ten patients underwent serial electrophysiology study (EPS) with assessment of fastest 1:1 AP conduction. The median age at first and follow-up EPS was 0 (median 4 days) and 53 months. Median fastest 1:1 AP conduction was 255 ms at initial EPS and 275 ms at follow-up (P=0.24). The interval of time between studies had no influence on stability over time, nor was there any appreciable effect following changes in retrograde AP conduction. In conclusion, no patient displayed any marked shortening of 1:1 AP conduction over time.


Asunto(s)
Fascículo Atrioventricular Accesorio/diagnóstico , Fascículo Atrioventricular Accesorio/fisiopatología , Envejecimiento , Niño , Preescolar , Electrocardiografía , Femenino , Humanos , Lactante , Recién Nacido , Estudios Longitudinales , Masculino , Conducción Nerviosa , Reproducibilidad de los Resultados , Sensibilidad y Especificidad
11.
Am J Perinatol ; 32(13): 1240-6, 2015 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-26039890

RESUMEN

OBJECTIVE: Fetal tachyarrhythmias complicate 0.5% of pregnancies, with high morbidity and mortality. We hypothesized that maternal factors may predispose to fetal supraventricular tachycardia (SVT). STUDY DESIGN: We reviewed medical records of all 124 mothers who presented to the Vanderbilt Fetal Cardiology Clinic from 2004 to 2010 for fetal arrhythmias, excluding heart block. Maternal factors were compared between 28 fetuses with SVT and a control group of 112 fetuses screened for noncardiac conditions. The proportions were analyzed using chi-square or Fisher exact test for categorical variables and Wilcoxon rank sum test for continuous variables. RESULTS: Of maternal factors, thyroid disease was statistically significant compared with controls. Among mothers whose fetuses had SVT, 21% had thyroid disease (83% hypothyroidism) compared with 3% of controls (p < 0.001). CONCLUSION: In this cohort, the maternal thyroid disease was more common in fetuses with SVT compared with controls (odds ratio = 9.8, 95% confidence interval 2.3-42.3), suggesting closer screening for fetal arrhythmias and SVT in mothers with thyroid disease. Also, routine screening of thyroid functions and thyroid autoantibodies may be warranted in mothers of fetuses with SVT.


Asunto(s)
Enfermedades Fetales/epidemiología , Hipotiroidismo/epidemiología , Complicaciones del Embarazo/epidemiología , Taquicardia Supraventricular/epidemiología , Adulto , Arritmias Cardíacas/epidemiología , Complejos Atriales Prematuros/epidemiología , Ecocardiografía , Femenino , Enfermedades Fetales/diagnóstico por imagen , Edad Gestacional , Humanos , Hidropesía Fetal/epidemiología , Hipotiroidismo/sangre , Hipotiroidismo/tratamiento farmacológico , Embarazo , Estudios Retrospectivos , Factores de Riesgo , Taquicardia Supraventricular/diagnóstico por imagen , Enfermedades de la Tiroides/sangre , Enfermedades de la Tiroides/epidemiología , Tirotropina/sangre , Tiroxina/sangre , Tiroxina/uso terapéutico , Ultrasonografía Prenatal , Complejos Prematuros Ventriculares/epidemiología , Adulto Joven
12.
N Engl J Med ; 365(20): 1896-904, 2011 Nov 17.
Artículo en Inglés | MEDLINE | ID: mdl-22043968

RESUMEN

BACKGROUND: Adverse-event reports from North America have raised concern that the use of drugs for attention deficit-hyperactivity disorder (ADHD) increases the risk of serious cardiovascular events. METHODS: We conducted a retrospective cohort study with automated data from four health plans (Tennessee Medicaid, Washington State Medicaid, Kaiser Permanente California, and OptumInsight Epidemiology), with 1,200,438 children and young adults between the ages of 2 and 24 years and 2,579,104 person-years of follow-up, including 373,667 person-years of current use of ADHD drugs. We identified serious cardiovascular events (sudden cardiac death, acute myocardial infarction, and stroke) from health-plan data and vital records, with end points validated by medical-record review. We estimated the relative risk of end points among current users, as compared with nonusers, with hazard ratios from Cox regression models. RESULTS: Cohort members had 81 serious cardiovascular events (3.1 per 100,000 person-years). Current users of ADHD drugs were not at increased risk for serious cardiovascular events (adjusted hazard ratio, 0.75; 95% confidence interval [CI], 0.31 to 1.85). Risk was not increased for any of the individual end points, or for current users as compared with former users (adjusted hazard ratio, 0.70; 95% CI, 0.29 to 1.72). Alternative analyses addressing several study assumptions also showed no significant association between the use of an ADHD drug and the risk of a study end point. CONCLUSIONS: This large study showed no evidence that current use of an ADHD drug was associated with an increased risk of serious cardiovascular events, although the upper limit of the 95% confidence interval indicated that a doubling of the risk could not be ruled out. However, the absolute magnitude of such an increased risk would be low. (Funded by the Agency for Healthcare Research and Quality and the Food and Drug Administration.).


Asunto(s)
Trastorno por Déficit de Atención con Hiperactividad/tratamiento farmacológico , Enfermedades Cardiovasculares/inducido químicamente , Estimulantes del Sistema Nervioso Central/efectos adversos , Adolescente , Trastorno por Déficit de Atención con Hiperactividad/complicaciones , Enfermedades Cardiovasculares/epidemiología , Niño , Preescolar , Estudios de Cohortes , Femenino , Humanos , Masculino , Estudios Retrospectivos , Riesgo , Adulto Joven
13.
Am J Cardiol ; 204: 14-21, 2023 10 01.
Artículo en Inglés | MEDLINE | ID: mdl-37536198

RESUMEN

Patients with repaired tetralogy of Fallot are at elevated risk for ventricular arrhythmia and sudden cardiac death. Over the past decade, the pathogenesis and natural history of ventricular tachycardia has become increasingly understood, and catheter ablation has emerged as an effective treatment modality. Concurrently, there has been great progress in the development of a versatile array of transcatheter valves that can be placed in the native right ventricular outflow tract for the treatment of long-standing pulmonary regurgitation. Although such valve platforms may eliminate the need for repeat cardiac operations, they may also impede catheter access to the myocardial substrates responsible for sustained macro-reentrant ventricular tachycardia. This manuscript provides the rationale and design of a recently devised multicenter study that will examine the clinical outcomes of a uniform, preemptive strategy to eliminate ventricular tachycardia substrates before transcatheter pulmonary valve implantation in patients with tetralogy of Fallot.


Asunto(s)
Ablación por Catéter , Implantación de Prótesis de Válvulas Cardíacas , Insuficiencia de la Válvula Pulmonar , Válvula Pulmonar , Taquicardia Ventricular , Tetralogía de Fallot , Humanos , Tetralogía de Fallot/complicaciones , Tetralogía de Fallot/cirugía , Válvula Pulmonar/cirugía , Arritmias Cardíacas , Insuficiencia de la Válvula Pulmonar/cirugía , Resultado del Tratamiento , Ablación por Catéter/efectos adversos , Implantación de Prótesis de Válvulas Cardíacas/efectos adversos
14.
Curr Opin Pediatr ; 24(5): 592-602, 2012 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-22954955

RESUMEN

PURPOSE OF REVIEW: Sudden death is a leading cause of death in industrialized nations. Sudden death in children represents a relatively small proportion of sudden death in the population, but has devastating effects on families and communities, and often attracts significant attention. RECENT FINDINGS: Several recent studies show that a portion of previously unexplained sudden death in children is due to cardiac conditions which are potentially identifiable by 'molecular autopsy' or investigation of family members. As some of these conditions that may present with sudden death can be detected by ECG, the controversial role of screening asymptomatic children will be discussed, as will the question of increased risk associated with stimulant medications. Recent pharmacologic and nonpharmacologic advancements for those with identifiable conditions at increased risk of sudden death will be reviewed. SUMMARY: Recent developments have refined our understanding of the various causes of sudden death in children. Post-mortem genetic testing and/or investigation of family members can be fruitful in determining a cause and identifying at-risk relatives. Given the available and potential treatments for specific disorders with increased risk of sudden death, the benefit of identifying such disorders early is clear. The debate regarding universal ECG screening is social and political, as well as scientific.


Asunto(s)
Arritmias Cardíacas/diagnóstico , Atletas/estadística & datos numéricos , Trastorno por Déficit de Atención con Hiperactividad/tratamiento farmacológico , Estimulantes del Sistema Nervioso Central/efectos adversos , Muerte Súbita/patología , Adolescente , Arritmias Cardíacas/mortalidad , Trastorno por Déficit de Atención con Hiperactividad/epidemiología , Autopsia , Causas de Muerte , Estimulantes del Sistema Nervioso Central/administración & dosificación , Niño , Preescolar , Muerte Súbita/epidemiología , Muerte Súbita/prevención & control , Electrocardiografía , Femenino , Pruebas Genéticas , Humanos , Lactante , Recién Nacido , Masculino , Tamizaje Masivo , Factores de Riesgo , Estados Unidos/epidemiología
15.
Europace ; 14(10): 1428-32, 2012 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-22562657

RESUMEN

AIM: The Kir 6.1 K(atp) channel is believed to play an important role in ventricular repolarization as determined from both functional and genetic studies of the potassium inwardly-rectifying channel, subfamily J, member 8 (KCNJ8)-S422L missense mutation in patients with J-wave syndromes. Although Kir6.1 is also present in atrial tissue, it is unknown whether this channel modulates atrial repolarization and hence whether the S422L mutation portends a greater risk of atrial arrhythmias. This study sought to examine whether there was an increased frequency of the KCNJ8-S422L mutation among patients with atrial fibrillation (AF) and early repolarization (ER) as a possible novel susceptibility gene for AF. METHODS AND RESULTS: A total of 325 lone AF probands were identified from the Vanderbilt AF Registry, a collection of clinical data and DNA from consented, consecutively enrolled participants. The coding regions of KCNJ8 were sequenced, and the patient's presenting electrocardiogram (ECG) was reviewed by two independent physicians for ER abnormalities. The KCNJ8-S422L mutation was identified in two AF probands while no other candidate gene variants were identified in these cases. Twenty-two (7%) patients were found to have ER on the ECG, including the two probands carrying the S422L variant. In one small AF kindred, the S422L variant co-segregated with AF and ER. CONCLUSIONS: The KCNJ8-S422L variant is associated with both increased AF susceptibility and ER indicating a role for Kir 6.1 K(atp) channel in both ventricular and atrial repolarization.


Asunto(s)
Fibrilación Atrial/genética , Fibrilación Atrial/fisiopatología , Canales KATP/genética , Adulto , Secuencia de Bases , Electrocardiografía , Femenino , Predisposición Genética a la Enfermedad , Indicadores de Salud , Humanos , Masculino , Persona de Mediana Edad , Datos de Secuencia Molecular , Mutación , Análisis de Secuencia de ADN , Adulto Joven
16.
Can J Cardiol ; 38(7): 1036-1047, 2022 07.
Artículo en Inglés | MEDLINE | ID: mdl-35240252

RESUMEN

Atrial arrhythmias are highly prevalent in the aging Fontan population and contribute importantly to morbidity and mortality. Although the most common arrhythmia is scar-based intra-atrial re-entrant tachycardia, various other arrhythmias may occur, including focal atrial tachycardia, atrioventricular node-dependent tachycardias, and atrial fibrillation. The type and prevalence of atrial arrhythmia is determined, in part, by the underlying congenital defect and variant of Fontan surgery. Although the cumulative incidence of atrial tachyarrhythmias has decreased substantially from the atriopulmonary anastomosis to the more recent total cavopulmonary-connection Fontan, the burden of atrial arrhythmias remains substantial. Management is often multifaceted and can include anticoagulation, anti-arrhythmic drug therapy, pacing, and cardioversion. Catheter ablation plays a key role in control of arrhythmia. Risks and benefits must be carefully weighed. Among the important considerations are the clinical burden of arrhythmia, ventricular function, hemodynamic stability in tachycardia, suspected arrhythmia mechanisms, risks associated with anaesthesia, venous access, approaches to reaching the pulmonary venous atrium, and accompanying comorbidities. Careful review of surgical notes, electrocardiographic tracings, and advanced imaging is paramount, with particular attention to anatomic abnormalities such as venous obstructions and displaced conduction systems. Despite numerous challenges, ablation of atrial arrhythmias is effective in improving clinical status. Nevertheless, onset of new arrhythmias is common during long-term follow-up. Advanced technologies, such as high-density mapping catheters and remote magnetic guided ablation, carry the potential to further improve outcomes. Fontan patients with atrial arrhythmias should be referred to centres with dedicated expertise in congenital heart disease including catheter ablation, anaesthesia support, and advanced imaging.


Asunto(s)
Fibrilación Atrial , Ablación por Catéter , Procedimiento de Fontan , Cardiopatías Congénitas , Taquicardia Supraventricular , Fibrilación Atrial/complicaciones , Ablación por Catéter/métodos , Procedimiento de Fontan/efectos adversos , Sistema de Conducción Cardíaco , Humanos , Taquicardia , Taquicardia Supraventricular/etiología
17.
Clin Transl Sci ; 15(7): 1787-1795, 2022 07.
Artículo en Inglés | MEDLINE | ID: mdl-35514162

RESUMEN

Propafenone is an antiarrhythmic drug metabolized primarily by cytochrome P450 2D6 (CYP2D6). In adults, propafenone adverse events (AEs) are associated with CYP2D6 poor metabolizer status; however, pediatric data are lacking. Subjects were tested for 10 CYP2D6 allelic variants and copy number status, and activity scores assigned to each genotype. Seventy-six individuals (median 0.3 [range 0-26] years old) were included. Propafenone AEs occurred in 29 (38%); 14 (18%) required drug discontinuation due to AE. The most common AEs were QRS (n = 10) and QTc (n = 6) prolongation. Those with AEs were older at the time of propafenone initiation (1.58 [0.13-9.92] vs. 0.20 [0.08-2.01] years old; p = 0.042). CYP2D6 activity scores were not associated with presence of an AE (odds ratio [OR] 0.48 [0.22-1.03]; p = 0.055) but with the total number of AE (ß1  = -0.31 [-0.60, -0.03]; p = 0.029), systemic AEs (OR 0.33 [0.13-0.88]; p = 0.022), and drug discontinuation for systemic AEs (OR 0.28 [0.09-0.83]; p = 0.017). Awareness of CYP2D6 activity score and patient age may aid in determining an individual's risk for an AE with propafenone administration.


Asunto(s)
Antiarrítmicos , Citocromo P-450 CYP2D6 , Síndrome de QT Prolongado , Propafenona , Adolescente , Adulto , Alelos , Antiarrítmicos/efectos adversos , Niño , Preescolar , Citocromo P-450 CYP2D6/genética , Variación Genética , Genotipo , Humanos , Lactante , Recién Nacido , Síndrome de QT Prolongado/inducido químicamente , Síndrome de QT Prolongado/genética , Propafenona/efectos adversos , Adulto Joven
18.
JACC Clin Electrophysiol ; 8(3): 322-330, 2022 03.
Artículo en Inglés | MEDLINE | ID: mdl-34600852

RESUMEN

OBJECTIVES: This study sought to describe the electrophysiologic properties and catheter ablation outcomes for atrioventricular reciprocating tacchycardia via twin atrioventricular nodes (T-AVRT). BACKGROUND: Although catheter ablation for T-AVRT is an established entity, there are few data on the electrophysiological properties and outcomes of this procedure. METHODS: An international, multicenter study was conducted to collect retrospective procedural and outcomes data for catheter ablation of T-AVRT. RESULTS: Fifty-nine patients with T-AVRT were identified (median age at procedure, 8 years [interquartile range: 4.4-17.0 years]; 49% male). Of these, 55 (93%) were diagnosed with heterotaxy syndrome (right atrial isomerism in 39, left atrial isomerism in 8, and indeterminate in 8). Twenty-three (39%) had undergone Fontan operation (12 extracardiac, 11 lateral tunnel). After the Fontan operation, atrial access was conduit or baffle puncture in 15 (65%), fenestration in 5 (22%), and retrograde in 3 (13%). Acute success was achieved in 43 (91%) of 47 attempts (targeting an anterior node in 23 and posterior node in 24). There was no high-grade AV block or change in QRS duration. Over a median of 3.8 years, there were 3 recurrences. Of 7 patients with failed index procedure or recurrent T-AVRT, 6 (86%) were associated with anatomical hurdles such as prior Fontan or catheter course through an interrupted inferior vena cava-to-azygous vein continuation (P = 0.11). CONCLUSIONS: T-AVRT can be targeted successfully with low risk for recurrence. Complications were rare in this population. Anatomical challenges were common among patients with reduced short and long-term efficacy, representing opportunities for improvement in procedural timing and planning.


Asunto(s)
Cardiomiopatías , Ablación por Catéter , Procedimiento de Fontan , Taquicardia Reciprocante , Nodo Atrioventricular , Ablación por Catéter/efectos adversos , Femenino , Humanos , Masculino , Estudios Retrospectivos
19.
Pacing Clin Electrophysiol ; 33(4): 469-74, 2010 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-20015126

RESUMEN

BACKGROUND: Studies in adults suggest that after entrainment from the right ventricle, a post-pacing interval (PPI) minus tachycardia cycle length (TCL), when corrected for atrioventricular node delay (cPPI-TCL), is useful to distinguish atrioventricular nodal reentry tachycardia (AVNRT) from orthodromic reciprocating tachycardia (ORT), but this has not been evaluated in children. METHODS: In 100 children undergoing catheter ablation, entrainment of ORT or AVNRT was performed from the right ventricular apex. The atrial-His (AH) interval was measured on the return cycle (post-AH) and during tachycardia just prior to pacing (pre-AH). The cPPI-TCL was calculated as (PPI-TCL) - (post-AH - pre-AH). In the first 50 children, the best cutoff was identified and then validated in the next 50 children. RESULTS: In the first 50 children, cPPI-TCL was longer in AVNRT compared with ORT (122 +/- 19 ms vs 63 +/- 23 ms, P < 0.001). Furthermore, cPPI-TCL exceeded 95 ms in all AVNRT patients, but was less than < 95 ms in 28 of 29 ORT patients. In the next 50 children, a cPPI-TCL < 95 ms was 100% specific for ORT; a cPPI-TCL > 95 ms was 95% specific for AVNRT. There was even greater separation of cPPI-TCL values comparing AVNRT with ORT utilizing a septal accessory pathway. CONCLUSIONS: The cPPI-TCL is a useful technique to distinguish AVNRT from ORT in children. Our data suggest that in children a cPPI-TCL < 95 ms excludes AVNRT, while a value > 95 ms is rarely observed in ORT. This technique is particularly useful to distinguish AVNRT from ORT utilizing a septal accessory pathway. (PACE 2010; 469-474).


Asunto(s)
Taquicardia por Reentrada en el Nodo Atrioventricular/diagnóstico , Taquicardia Reciprocante/diagnóstico , Adolescente , Ablación por Catéter , Niño , Diagnóstico Diferencial , Humanos , Estudios Prospectivos , Taquicardia por Reentrada en el Nodo Atrioventricular/fisiopatología , Taquicardia por Reentrada en el Nodo Atrioventricular/cirugía , Taquicardia Reciprocante/fisiopatología , Taquicardia Reciprocante/cirugía
20.
medRxiv ; 2020 Sep 02.
Artículo en Inglés | MEDLINE | ID: mdl-32908996

RESUMEN

Background Myocarditis is a leading cause of sudden cardiac death among competitive athletes and may occur without antecedent symptoms. COVID-19-associated myocarditis has been well-described, but the prevalence of myocardial inflammation and fibrosis in young athletes after COVID-19 infection is unknown. Objectives This study sought to evaluate the prevalence and extent of cardiovascular involvement in collegiate athletes that had recently recovered from COVID-19. Methods We conducted a retrospective cohort analysis of collegiate varsity athletes with prior COVID-19 infection, all of whom underwent cardiac magnetic resonance (CMR) prior to resumption of competitive sports in August 2020. Results Twenty-two collegiate athletes with prior COVID-19 infection underwent CMR. The median time from SARS-CoV-2 infection to CMR was 52 days. The mean age was 20.2 years. Athletes represented 8 different varsity sports. This cohort was compared to 22 healthy controls and 22 tactical athlete controls. Most athletes experienced mild illness (N=17, 77%), while the remainder (23%) were asymptomatic. No athletes had abnormal troponin I, electrocardiograms, or LVEF < 50% on echocardiography. Late gadolinium enhancement was found in 9% of collegiate athletes and one athlete (5%) met formal criteria for myocarditis. Conclusions Our study suggests that the prevalence of myocardial inflammation or fibrosis after an asymptomatic or mild course of ambulatory COVID-19 among competitive athletes is modest (9%), but would be missed by ECG, Ti, and strain echocardiography. Future investigation is necessary to further phenotype cardiovascular manifestations of COVID-19 in order to better counsel athletes on return to sports participation.

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