Gennaio 2014 - Volume XXXIII - numero 1
Problemi speciali
1UCO Clinica Pediatrica, 2SS DPT Cardiologia, 3SC Pediatria d’urgenza e Pronto Soccorso Pediatrico,
IRCCS Materno-Infantile “Burlo Garofolo”, Trieste
4Scuola di Specializzazione in Pediatria - Seconda Università di Napoli
5Pediatra, ASL 9 Grosseto
Indirizzo per corrispondenza: giovanna.ferrara@gmail.com
Key words: Long QT syndrome, Congenital arrhythmia, Syncope, Drugs prolonging QT
The long QT syndrome (LQTS) is an arrhythmogenic syndrome due to cardiac ion channel disorders characterized by prolonged QT interval on ECG (QTc >440 ms for male, >460 ms for female) and the most common presentations are syncope, seizures, cardiac arrest and sudden death. Many different congenital forms have been identified but also an acquired form due to specific drugs, hypokalaemia, or hypomagnesemia is known. Familiarity is the leading risk factor. LQTS should be suspected in case of any syncope in order to perform ECG and start proper therapy. To identify and remove risk factors it is necessary to avoid potentially life-threatening arrhythmia in these patients.
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