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La tormenta tiroidea es un estado crítico y poco frecuente que condiciona la disfunción de múltiples órganos por el efecto del exceso de las hormonas tiroideas, esta disfunción endócrina tiene una elevada mortalidad y genera manifestaciones típicas como la taquicardia, fiebre, alteraciones gastrointestinales, cardiovasculares y del sistema nervioso central. El embarazo se ha asociado con un incremento en la incidencia de arritmias. Necesitan un tratamiento inmediato con drogas antiarrítmicas, cardioversión eléctrica o cesárea de urgencia. El WPW es una anormalidad cardiaca congénita que consiste en la presencia de un haz anómalo (Haz de Kent) que evita el sistema normal de conducción uniendo directamente aurículas y ventrículos. Veremos el caso de una gestante de 32 semanas que presenta un cuadro de tormenta tiroidea y múltiples episodios de taquicardia paroxística supraventricular (TPS), de tórpida y sombría evolución clínica mediada por un haz anómalo de Kent intermitente. Es evidente que la tormenta tiroidea en el contexto de la gestación produjo cambios en las propiedades electrofisiológicas del haz anómalo de Kent intermitente lo cual propició el desarrollo de múltiples taquicardias paroxísticas supraventriculares refractarias a la cardioversión eléctrica y farmacológica. Tampoco mejoró con la tiroidectomía total, solamente cedió por completo con la ablación por catéter de radiofrecuencia del haz anómalo de Kent.

Thyroid storm is a critical and infrequent state that conditions the dysfunction of multiple organs due to the effect of excess thyroid hormones. This endocrine dysfunction has a high mortality and generates typical manifestations such as tachycardia, fever, gastrointestinal, cardiovascular and heart disorders, and the central nervous system. Pregnancy has been associated with an increased incidence of arrhythmias. They need immediate treatment with antiarrhythmic drugs, electrical cardioversion, or emergency caesarean section. WPW is a congenital cardiac abnormality that consists of the presence of an abnormal bundle (Kent bundle) that prevents the normal conduction system, directly joining the atria and ventricles. We will see the case of a 32-week pregnant woman who presented symptoms of thyroid storm and multiple episodes of paroxysmal supraventricular tachycardia (PST), with a torpid clinical course mediated by an abnormal intermittent Kent bundle. It is evident that the thyroid storm in the context of pregnancy produced changes in the electrophysiological properties of the intermittent Kent bundle, which led to the development of multiple PST refractory to electrical and pharmacological cardioversion. Moreover, it also did not improve with total thyroidectomy, only resolved completely with radiofrequency catheter ablation of the Kent bundle.

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BACKGROUND: Coumel tachycardia is an infrequent form of supraventricular tachycardia (SVT) that usually occurs in infants and children. It is a tachycardia mediated by an accessory pathway with retrograde slow conduction that explains the classic ECG pattern with long RP' interval and negative P waves in leads II, III, and aVF. In this study, we describe the clinical course and management of Coumel tachycardia in children. CASE REPORT: We conducted a retrospective review of five consecutive pediatric patients, mean age 11 ± 3 years (range 6 to 14). The first episode of SVT was at a mean age of 10.4 ± 4.8 years (range 2 to 14) with a mean evolution of 7.4 ± 9.4 months (range 1 to 24). Pharmacological therapy was unsuccessful despite the combination of antiarrhythmic drugs. The tachycardia was incessant with a density > 85% by 24-hour Holter monitoring; one patient developed tachycardia-induced cardiomyopathy. All children underwent successful radiofrequency catheter ablation, mean 5 ± 3 applications (range 1 to 8) with a single session and with no complications. After a mean follow-up of 24 ± 16 months, all patients were asymptomatic and recurrence-free without antiarrhythmic treatment. CONCLUSIONS: Coumel tachycardia is clinically persistent and usually refractory to antiarrhythmic treatment with substantial risk of tachycardia-mediated cardiomyopathy. Catheter ablation is effective and safe in children; thus, it should be indicated promptly and based on individual selection.

INTRODUCCIÓN: La taquicardia de Coumel es una forma poco frecuente de taquicardia supraventricular que suele presentarse en lactantes. Es una taquicardia mediada por una vía accesoria de conducción lenta retrógrada que explica el patrón ECG clásico con intervalo RP' largo y ondas P negativas en las derivaciones II, III y aVF. En este trabajo se describe el curso clínico y el manejo de la taquicardia de Coumel en niños. CASO CLÍNICO: Se llevó a cabo una revisión retrospectiva de cinco pacientes pediátricos consecutivos, con una media de edad de 11 ± 3 años (intervalos 6 a 14). El primer episodio de taquicardia 10.4 ± 4.8 años con evolución de 7.4 ± 9.4 meses. El tratamiento farmacológico fue ineficaz a pesar de la combinación de antiarrítmicos. La taquicardia era incesante con una densidad > 85% por Holter-24h; un paciente desarrolló miocardiopatía inducida por taquicardia. Todos los niños fueron sometidos a ablación con catéter y radiofrecuencia con éxito, y un promedio de 5 ± 3 aplicaciones en una sola sesión y sin complicaciones. Después de un seguimiento de 24 ± 16 meses, todos los pacientes fueron asintomáticos y libres de recurrencia sin tratamiento antiarrítmico. CONCLUSIONES: La taquicardia de Coumel es clínicamente persistente y generalmente refractaria al tratamiento antiarrítmico con un riesgo sustancial de miocardiopatía mediada por taquicardia. La ablación con catéter es eficaz y segura en niños, por lo que debe indicarse de forma temprana y en lactantes de una selección individual.

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BACKGROUND: The differential diagnosis between orthodromic atrioventricular reentry tachycardia (AVRT) and atypical AV nodal reentrant tachycardia (aAVNRT) is sometimes challenging. We hypothesize that aAVNRTs have more variability in the retrograde conduction time at tachycardia onset than AVRTs. METHODS: We aimed to assess the variability in retrograde conduction time at tachycardia onset in AVRT and aAVNRT and to propose a new diagnostic tool to differentiate these two arrhythmia mechanisms. We measured the VA interval of the first beats after tachycardia induction until it stabilized. The difference between the maximum and minimum VA intervals (∆VA) and the number of beats needed for the VA interval to stabilize was analyzed. Atrial tachycardias were excluded. RESULTS: A total of 107 patients with aAVNRT (n = 37) or AVRT (n = 64) were included. Six additional patients with decremental accessory pathway-mediated tachycardia (DAPT) were analyzed separately. All aAVNRTs had VA interval variability. The median ∆VA was 0 (0 - 5) ms in AVRTs vs 40 (21 - 55) ms in aAVNRTs (p < 0.001). The VA interval stabilized significantly earlier in AVRTs (median 1.5 [1 - 3] beats) than in aAVNRTs (5 [4 - 7] beats; p < 0.001). A ∆VA < 10 ms accurately differentiated AVRT from aAVNRT with 100% of sensitivity, specificity, and positive and negative predictive values. The stabilization of the VA interval at < 3 beats of the tachycardia onset identified AVRT with sensitivity, specificity, and positive and negative predictive values of 64.1%, 94.6%, 95.3%, and 60.3%, respectively. A ∆VA < 20 ms yielded good diagnostic accuracy for DAPT. CONCLUSIONS: A ∆VA < 10 ms is a simple and useful criterion that accurately distinguished AVRT from atypical AVNRT. Central panel: Scatter plot showing individual values of ∆VA in atypical AVNRT and AVRT. Left panel: induction of atypical AVNRT. The VA interval stabilizes at the 5th beat and the ∆VA is 62 ms (maximum VA interval: 172 ms - minimum VA interval: 110 ms). Right panel: induction of AVRT. The tachycardia has a fixed VA interval from the first beat. ∆VA is 0 ms.

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Abstract Background: Coumel tachycardia is an infrequent form of supraventricular tachycardia (SVT) that usually occurs in infants and children. It is a tachycardia mediated by an accessory pathway with retrograde slow conduction that explains the classic ECG pattern with long RP' interval and negative P waves in leads II, III, and aVF. In this study, we describe the clinical course and management of Coumel tachycardia in children. Case report: We conducted a retrospective review of five consecutive pediatric patients, mean age 11 ± 3 years (range 6 to 14). The first episode of SVT was at a mean age of 10.4 ± 4.8 years (range 2 to 14) with a mean evolution of 7.4 ± 9.4 months (range 1 to 24). Pharmacological therapy was unsuccessful despite the combination of antiarrhythmic drugs. The tachycardia was incessant with a density > 85% by 24-hour Holter monitoring; one patient developed tachycardia-induced cardiomyopathy. All children underwent successful radiofrequency catheter ablation, mean 5 ± 3 applications (range 1 to 8) with a single session and with no complications. After a mean follow-up of 24 ± 16 months, all patients were asymptomatic and recurrence-free without antiarrhythmic treatment. Conclusions: Coumel tachycardia is clinically persistent and usually refractory to antiarrhythmic treatment with substantial risk of tachycardia-mediated cardiomyopathy. Catheter ablation is effective and safe in children; thus, it should be indicated promptly and based on individual selection.

Resumen Introducción: La taquicardia de Coumel es una forma poco frecuente de taquicardia supraventricular que suele presentarse en lactantes. Es una taquicardia mediada por una vía accesoria de conducción lenta retrógrada que explica el patrón ECG clásico con intervalo RP' largo y ondas P negativas en las derivaciones II, III y aVF. En este trabajo se describe el curso clínico y el manejo de la taquicardia de Coumel en niños. Caso clínico: Se llevó a cabo una revisión retrospectiva de cinco pacientes pediátricos consecutivos, con una media de edad de 11 ± 3 años (intervalos 6 a 14). El primer episodio de taquicardia 10.4 ± 4.8 años con evolución de 7.4 ± 9.4 meses. El tratamiento farmacológico fue ineficaz a pesar de la combinación de antiarrítmicos. La taquicardia era incesante con una densidad > 85% por Holter-24h; un paciente desarrolló miocardiopatía inducida por taquicardia. Todos los niños fueron sometidos a ablación con catéter y radiofrecuencia con éxito, y un promedio de 5 ± 3 aplicaciones en una sola sesión y sin complicaciones. Después de un seguimiento de 24 ± 16 meses, todos los pacientes fueron asintomáticos y libres de recurrencia sin tratamiento antiarrítmico. Conclusiones: La taquicardia de Coumel es clínicamente persistente y generalmente refractaria al tratamiento antiarrítmico con un riesgo sustancial de miocardiopatía mediada por taquicardia. La ablación con catéter es eficaz y segura en niños, por lo que debe indicarse de forma temprana y en lactantes de una selección individual.

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The present case contributes to understanding the mechanism and differential diagnosis of a wide QRS complex tachycardia with varying degrees of right bundle branch block morphology.

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La taquicardia supraventricular (TSV) es la principal taquiarritmia en el recién nacido (RN) que requiere una resolución urgente. Por su parte, la enterocolitis necrosante (ECN) es la emergencia gastrointestinal más común que afecta principalmente a RN prematuros. Aunque estas se reconocen como patologías distintas, la bibliografía sugiere que los episodios de TSV pueden predisponer a los pacientes a la ECN a través de alteraciones en el flujo sanguíneo mesentérico y una disminución de la perfusión tisular. Se presenta aquí el caso clínico de un neonato prematuro que desarrolló un cuadro de ECN luego de un evento aislado de TSV con bajo gasto cardíaco

Supraventricular tachycardia (SVT) is the main tachyarrhythmia in the newborn (NB) that requires urgent resolution. Necrotizing enterocolitis (NEC) is the most common gastrointestinal emergency that mainly affects premature infants. Although these conditions are recognized as distinct pathologies, literature reports suggest that episodes of SVT may predispose patients to NEC secondary to disturbances in mesenteric blood flow and a decrease in tissue perfusion. We present here the clinical case of a premature neonate who developed NEC after an isolated SVT event with low cardiac output

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Supraventricular tachycardia (SVT) is the main tachyarrhythmia in the newborn (NB) that requires urgent resolution. Necrotizing enterocolitis (NEC) is the most common gastrointestinal emergency that mainly affects premature infants. Although these conditions are recognized as distinct pathologies, literature reports suggest that episodes of SVT may predispose patients to NEC secondary to disturbances in mesenteric blood flow and a decrease in tissue perfusion. We present here the clinical case of a premature neonate who developed NEC after an isolated SVT event with low cardiac output.

La taquicardia supraventricular (TSV) es la principal taquiarritmia en el recién nacido (RN) que requiere una resolución urgente. Por su parte, la enterocolitis necrosante (ECN) es la emergencia gastrointestinal más común que afecta principalmente a RN prematuros. Aunque estas se reconocen como patologías distintas, la bibliografía sugiere que los episodios de TSV pueden predisponer a los pacientes a la ECN a través de alteraciones en el flujo sanguíneo mesentérico y una disminución de la perfusión tisular. Se presenta aquí el caso clínico de un neonato prematuro que desarrolló un cuadro de ECN luego de un evento aislado de TSV con bajo gasto cardíaco.

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BACKGROUND: Accessory pathway (AP)-related arrhythmias are frequent in patients with Ebstein anomaly (EA), and arrhythmia recurrence after catheter ablation remains high despite current technological developments. METHODS: Case series report of patients with EA who were taken to an accessory pathway ablation procedure and where clinical, procedure, and follow-up data are described. In all cases, mapping of the true tricuspid annulus guided by intracardiac ultrasound was used. RESULTS: Six patients with EA underwent an ablation procedure using ICE to delineate the true tricuspid annulus. The duration of the procedure was 253.33 ± 60.92 min, with an acute success of 100%. After a mean follow-up of 16.16 ± 7.7 months, no recurrences of tachycardia were documented, and all patients were free of antiarrhythmic medications. CONCLUSION: Intraprocedural ICE helps to delineate the true tricuspid annulus that contains the APs, facilitating mapping and ablation. We hypothesize that the systematic use of ICE in this scenario improves ablation efficacy while reducing complications, but this must be verified in prospective studies.

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Background: New techniques for cardiac arrhythmia ablation, such as contact force (CF) technology, have emerged recently. These catheters provide information about adequate tissue contact for optimal lesions. In adults, these techniques have shown greater accuracy, reduced arrhythmia recurrence and complications, and higher success rates. However, data on pediatric patients are limited. Objective: The purpose of this study was to describe the experience with arrhythmia ablation using CF catheters in the pediatric population. Methods: A retrospective cross-sectional descriptive study of all patients <18 years old undergoing cardiac 3-dimensional mapping and ablation with CF between March 2016 and June 2022 was performed. Results: A total of 321 patients were included (51.40% male; mean age 12.26 years). The most frequent arrhythmia were supraventricular tachycardia (SVT) mediated by accessory pathways (APs) (atrioventricular reentrant tachycardia in 82.24%, ventricular arrhythmia in 11.21%, atrial tachycardia in 5.92%). Mean procedural time was 2.86 ± 1.2 hours, and average contact used was 14.33g ± 6.88g. The success rate of ablation was 97.82% with a low risk of complications. Conclusion: This is the largest published series of CF technology use in patients <18 years old. In the pediatric population, CF ablation is a safe procedure with high success rates and can be used for most arrhythmic substrates. The most frequent tachycardia observed in this study was SVT mediated by APs. Contact with 14g is safe and yields an excellent outcome in children. The presence of structural heart anomalies and previous ablation procedures decreased the success rate.

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BACKGROUND/OBJECTIVES: Implantable cardioverter defibrillators are used to prevent sudden cardiac death. The subcutaneous implantable cardioverter-defibrillator was newly developed to overcome the limitations of the conventional implantable cardioverter defibrillator-transvenous device. The subcutaneous implantable cardioverter defibrillator is indicated for young patients with heart disease, congenital heart defects, and poor venous access, who have an indication for implantable cardioverter defibrillator without the need for anti-bradycardic stimulation. We aimed to compare the efficacy and complications of subcutaneous with transvenous implantable cardioverter- defibrillator devices. METHODOLOGY: A systematic review was conducted using different databases. The inclusion criteria were observational and clinical randomized trials with no language limits and no publication date limit that compared subcutaneous with transvenous implantable cardioverter-defibrillators. The selected patients were aged > 18 years with complex ventricular arrhythmia. RESULTS: Five studies involving 2111 patients who underwent implantable cardioverter defibrillator implantation were included. The most frequent complication in the subcutaneous device group was infection, followed by hematoma formation and electrode migration. For the transvenous device, the most frequent complications were electrode migration and infection. Regarding efficacy, the total rates of appropriate shocks were 9.04% and 20.47% in the subcutaneous and transvenous device groups, respectively, whereas inappropriate shocks to the subcutaneous and transvenous device groups were 11,3% and 10,7%, respectively. CONCLUSION: When compared to the transvenous device, the subcutaneous device had lower complication rates owing to lead migration and less inappropriate shocks due to supraventricular tachycardia; nevertheless, infection rates and improper shocks due to T wave oversensing were comparable for both devices CRD42021251569.

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