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Abstract
A síndrome do QT longo congênito representa importante distúrbio genético, e está associada a síncope, parada cardíaca e morte súbita. O diagnóstico é baseado principalmente na medida do intervalo QT corrigido associada a critérios clínicos e história familiar. A estratificaçao de risco auxilia na decisao terapêutica. Relatamos o caso de uma paciente com síndrome do QT longo congênito, portadora de marcapasso definitivo e nefropatia espoliadora de magnésio, que evoluiu com necessidade de upgrade para cardiodesfibrilador implantável na ocasiao da troca do gerador do dispositivo.
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