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ISSN 0035-0052 (Impreso)

2008, Número 3

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Rev Mex Pediatr 2008; 75 (3)


Síndrome de QT largo en pediatría

Gómez-Gómez M, Danglot-Banck C, Santamaría-Díaz H

Idioma: Español
Referencias bibliográficas: 50
Paginas: 121-131
Archivo PDF: 161.76 Kb.


RESUMEN

El síndrome identificado por electrocardiograma como un QT largo y manifestaciones clínicas puede ser de origen congénito o adquirido y se le encuentra asociado con sordera congénita; se le conoce como síndrome de Jervell y Lange-Nielsen. Cuando no hay sordera se identifica como síndrome de Romano-Ward. En este reporte se hace una revisión de los mecanismos moleculares y su correlación con la clínica, y se mencionan sus particularidades clínicas y los factores de riesgo asociados: sordera congénita, sexo femenino, presentación a una edad temprana, antecedente de síncope, duración del QTc mayor a 500 ms y torsades des pointes. La mortalidad por este padecimiento ha disminuido a menos de 10% con el empleo de beta (β) bloqueadores y la implantación de un marcapaso o de un desfibrilador automático. Se incluye un algoritmo de manejo de estos pacientes.


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