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oai:repisalud.isciii.es:20.500.12105/140112025-03-10T15:49:04Zcom_20.500.12105_5571com_20.500.12105_2404com_20.500.12105_2403col_20.500.12105_5710

00925njm 22002777a 4500 dc Centeno Malfaz, F author Bello Martínez, B author Beltrán Pérez, AI author Alcalde Martín, C author López García C author 2006-10 The long QT syndrome (LQTS) is an hereditary disease that produces a malfunction on the sodium and potassium channels of the heart and lengthens the duration of the cardiac repolarization stage. It is characterized by the appearance of syncopes, arrhythmias and even sudden death. There are two variants of the congenital LQTS: the autosomal dominant Romano-Ward syndrome and the autosomal recessive Jervell-Lange-Nielsen syndrome, that is associated with sensorineural deafness and is less frequent than the other type. The diagnosis is made from the clinical criteria, the electrocardiogram and the family history. In the last few years, molecular studies have been developed, opening new possibilities not only for its diagnosis but also for the treatment of these patients. Boletín del ECEMC: Rev Dismor Epidemiol 2006; V (nº 5): 84-90 0210–3893 Boletín del ECEMC: Revista de Dismorfología y Epidemiología http://hdl.handle.net/20.500.12105/14011 Síndrome de intervalo QT largo congénito