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Quick Scroll congenital prolonged qt syndrome 11.12.05 (2 years ago) #1
Congenital Long QT syndrome

Jervell-Lange-Nielson syndrome is inherited in an autosomal recessive manner and is associated with deafness. Romano-Ward syndrome is autosomal dominant. Approx 10% of affected individuals have a normal QT interval.
3 different mutations have been described in patients with congenital long QT syndrome:-
Chr 3 - sodium channel;
Chr 7 - potassium channel;
Chr 11 - mutation unknown;

Beta-blocker therapy, cervical sympathectomy (left more often than right) and prophylactic pacing reduce the risk of syncopal episodes.

Bad prognostic features are:-
• Positive family history of syncope or sudden death
• QTc > 0.54 sec
• Congenital deafness
• AV block
• Documented ventriculat arrhythmias

Other causes of long QT interval include hypocalcaemia, hypomagnesaemia and drugs such as quinidine, disopyramide, amiodarone, sotalol, terfenadine, and chlorpromazine.

Patients should be reviewed by an cardiac electrophysiologist and assessed for the need for an Automatic Implantable Cardio Defibrillator (AICD)
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