Rivista di formazione e aggiornamento professionale del pediatra e del medico di base, fondata nel 1982. In collaborazione con l'Associazione Culturale Pediatri.
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Problemi speciali

La sindrome del QT lungo

The long QT syndrome

Giovanna Ferrara1, Emanuela Berton2, Claudio Germani3, Pierluigi Marzuillo4, Paolo Bonazza5, Raffaele D’alfonso5, Egidio Barbi3

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

Gennaio 2014 - pagg. 33 -40

Abstract
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.
Parole chiave
aritmia congenita Congenital arrhythmia Drugs prolonging QT Farmaci che prolungano l'intervallo QT Long QT syndrome sincope Sindrome del QT lungo Syncope
Suggerite dall'AI
Sudden cardiac death
Classificazione MeSH
Chemical Actions and Uses Pharmacologic Actions Therapeutic Uses Cardiovascular Agents Anti-Arrhythmia Agents (7)
Pathological Conditions, Signs and Symptoms Pathologic Processes Death Death, Sudden Death, Sudden, Cardiac (18)
Diagnosis Diagnostic Techniques and Procedures Diagnostic Techniques, Cardiovascular Heart Function Tests Electrocardiography (44)
Amino Acids, Peptides, and Proteins Proteins Carrier Proteins Membrane Transport Proteins Ion Channels (2)
Congenital, Hereditary, and Neonatal Diseases and Abnormalities Congenital Abnormalities Cardiovascular Abnormalities Heart Defects, Congenital Long QT Syndrome Jervell-Lange Nielsen Syndrome
Congenital, Hereditary, and Neonatal Diseases and Abnormalities Congenital Abnormalities Cardiovascular Abnormalities Heart Defects, Congenital Long QT Syndrome (18)
Congenital, Hereditary, and Neonatal Diseases and Abnormalities Congenital Abnormalities Cardiovascular Abnormalities Heart Defects, Congenital Long QT Syndrome Romano-Ward Syndrome
Pathological Conditions, Signs and Symptoms Signs and Symptoms Neurologic Manifestations Neurobehavioral Manifestations Consciousness Disorders Unconsciousness
Cardiovascular Diseases Heart Diseases Arrhythmias, Cardiac Tachycardia Tachycardia, Ventricular Torsades de Pointes (3)
Leggi anche
RI Il commento
Attenti a quelle due
Bronzetti G. · 2022
Jervell-Lange Nielsen Syndrome/Arrhythmias, Cardiac, Romano-Ward Syndrome/Arrhythmias, Cardiac, Death, Sudden, Cardiac, Long QT Syndrome/Arrhythmias, Cardiac
RI Domande e risposte
QT lungo, farmaci a rischio aritmico Amoxicillina + acido clavulanico, dosaggio aumentato
· 2021
Jervell-Lange Nielsen Syndrome/Long QT Syndrome, Romano-Ward Syndrome/Long QT Syndrome, Torsades de Pointes, Long QT Syndrome
RI Aggiornamento
La sincope in età pediatrica
Corona F, Tessitore A, Bobbo M, Barbi E, Cozzi G. · 2022
Jervell-Lange Nielsen Syndrome/Arrhythmias, Cardiac, Romano-Ward Syndrome/Arrhythmias, Cardiac, Long QT Syndrome/Arrhythmias, Cardiac, Syncope
RI Percorsi clinici
Tante sincopi con l’angoscia di correre
M. Copertino, F. Marchetti, A. Benettoni, et al. · 2009
Jervell-Lange Nielsen Syndrome/Long QT Syndrome, Romano-Ward Syndrome/Long QT Syndrome, Torsades de Pointes/Tachycardia, Ventricular, Death, Sudden, Cardiac
EL I Poster degli specializzandi
QT lungo congenito ed epilessia: quale relazione?
L. Cortellazzo Wiel · 2019
Jervell-Lange Nielsen Syndrome/Arrhythmias, Cardiac, Romano-Ward Syndrome/Arrhythmias, Cardiac, Long QT Syndrome/Arrhythmias, Cardiac, Syncope
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Corrispondenza: giovanna.ferrara@gmail.com