ABSTRACT
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 requires the application of the otolaryngologist specialist's own measures. The cardiovascular management implies the modification of lifestyles, mainly the prohibition of competitive sports, including swimming, avoiding exposure to loud sounds or triggers. The medications used include beta-blockers, and more rarely flecainide, ranozaline, and verapamil; invasive management consists of the implantation of a cardioverter defibrillator or even left sympathetic denervation, each with its own risks and benefits. In any of the cases, we must avoid the circumstances that increase the QT interval, as well as carry out the appropriate analysis of the benefits and risks of each possible invasive measure.
ABSTRACT
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 at least one hidden accessory pathway, 5% corresponded to permanent reciprocating junctional tachycardia, 5% intranodal tachycardia, 11.7% atrial tachycardia, 10 % atrial flutter, 5% had other types of tachycardia and in 5 cases no tachycardia was induced. Of the total procedures, 76% corresponded to electrophysiological study and ablation, four patients recurred. Conclusions: The most common supraventricular tachycardia substrate in children was the presence of some accessory pathway. Radiofrequency catheter ablation could be applied in children with a high success rate and a low complication rate.
ABSTRACT
Introducción: Los defectos de la pared abdominal anterior (onfalocele y gastrosquisis) conforman un espectro de malformaciones anatómicas estructurales de etiología diversa con severidad y pronóstico variable. Su incidencia es de 1:3000 a 5000 recién nacidos vivos. En los últimos años se ha evidenciado un aumento en la prevalencia de gastrosquisis, asociado con una creciente proporción de madres adolescentes y a nuevos factores etiológicos como exposición a teratógenos específicos y deficiencias nutricionales. Objetivo: Conocer las características clínicas y epidemiológicas de recién nacidos con onfalocele y gastrosquisis; y los resultados del manejo de estos pacientes, en un hospital nacional. Material y métodos: Se analizaron las historias clínicas de 15 neonatos en total, 12 con gastrosquisis y 3 con onfalocele, atendidos en el hospital en un lapso de tres años. Resultados: La prevalencia de los defectos de pared abdominal fue 14.67/10,000 nacidos vivos, con una frecuencia relativa de 2.97 y 11.90/10,000 nacidos vivos para onfalocele y gastrosquisis respectivamente. Los niños con gastrosquisis pesaron al nacer 2367.9±491 g y los de onfalocele 2040±1712 g; y la edad de gestación fue similar, 8 fueron sometidos a cesárea, mientras que 7 a parto vaginal; la permanencia en UCIN fue de 16 días en promedio, 9 para los casos de onfalocele y 18.6 para gastrosquisis. Siete murieron: cinco con gastrosquisis y dos con onfalocele. Conclusiones: Los factores asociados a estas enfermedades fueron similares a lo informado por otros. La mortalidad es aún alta por lo que se hacen cambios en el manejo de estos neonatos.
Introduction: Abdominal wall defects (omphalocele and gastroschisis) represent a spectrum of structural anatomical malformations with several etiology and variable prognosis. The incidence is 1: 3000 to 5000 live births. In the last years has been detected an increase of the prevalence, associated to an increasing proportion of teenage mothers and to new etiologic factors like exposition to specific teratogens and nutritional deficiencies. Objective: To know the clinical features and the neonatal survival of infants with gastroschisis and omphalocele in a general hospital. Subjects and methods: The hospital files of 12 neonates with gastroschisis and three with omphalocele during three years were review. Results: The prevalence of abdominal wall defects was 14.67/10,000 live births, with a relative frequency of 2.97/10,000 live births for omphalocele and 11.90/10,000 live births for gastroschisis. The birth weight of patients with gastroschisis was 2367.9±491 g and the other with omphalocele was 2040±1712 g they have similar gestational age. There were 8 cesarean section, whereas 7 vaginal delivery; stay in PICU were 16 days, 9 for the cases of omphalocele and 18.6 for gastroschisis. Seven of these neonates died, five with gastroschisis and two with omphalocele. Conclusions: The features of the neonates studied were similar to other reported. The mortality is still high but we are realized measures to respect.
