Congenital long QT syndrome (LQTS) represents a group of heart diseases of genetic origin characterized by prolongation of the QT interval and an abnormal T wave on the electrocardiogram (ECG). They can have a dominant or recessive expression, the latter associated with sensorineural deafness. In both cases, its clinical presentation is associated with recurrent syncope and sudden death as a consequence of ventricular tachycardia, specifically Torsades de Pointes. Currently they are classified according to the specific genetic defect, being able to compromise around 16 genes and almost 2000 mutations. It should be suspected in individuals with related symptoms, electrocardiographic findings, and family history. Management is based on the reduction or elimination of symptoms, and concomitantly the prevention of sudden death (SD), in those children with congenital deafness, the management ...

Congenital long QT syndrome (LQTS) represents a group of heart diseases of genetic origin characterized by prolongation of the QT interval and an abnormal T wave on the electrocardiogram (ECG). They can have a dominant or recessive expression, the latter associated with sensorineural deafness. In both cases, its clinical presentation is associated with recurrent syncope and sudden death as a consequence of ventricular tachycardia, specifically Torsades de Pointes. Currently they are classified according to the specific genetic defect, being able to compromise around 16 genes and almost 2000 mutations. It should be suspected in individuals with related symptoms, electrocardiographic findings, and family history. Management is based on the reduction or elimination of symptoms, and concomitantly the prevention of sudden death (SD), in those children with congenital deafness, the management ...

Objective. This study aims to describe the clinical, electrophysiological and therapeutic characteristics of pediatric patients with supraventricular tachycardia undergoing radiofrequency ablation. Materials and methods. Observational, descriptive, retrospective study of children admitted for supraventricular tachycardia treatment at the Instituto Nacional de Salud del Niño during 2018 to 2021. Results. Data from 62 procedures were collected, corresponding to 59 patients (mean age: 9.91 years, 61% male), 23% of procedures were performed in patients under 15kg, and two were in patients under 5kg. 63% of patients had a structurally normal heart, while 37% had some type of congenital heart disease, the most common being Ebstein’s anomaly. The most widely used group of drugs were beta-blockers, mainly propranolol. Among the arrhythmias treated, 21.7% had preexcitation Syndrome, 33.3% had ...

Objective. This study aims to describe the clinical, electrophysiological and therapeutic characteristics of pediatric patients with supraventricular tachycardia undergoing radiofrequency ablation. Materials and methods. Observational, descriptive, retrospective study of children admitted for supraventricular tachycardia treatment at the Instituto Nacional de Salud del Niño during 2018 to 2021. Results. Data from 62 procedures were collected, corresponding to 59 patients (mean age: 9.91 years, 61% male), 23% of procedures were performed in patients under 15kg, and two were in patients under 5kg. 63% of patients had a structurally normal heart, while 37% had some type of congenital heart disease, the most common being Ebstein’s anomaly. The most widely used group of drugs were beta-blockers, mainly propranolol. Among the arrhythmias treated, 21.7% had preexcitation Syndrome, 33.3% had ...