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Q. nine year old boy is being evaluated for a syncopal episode. He was playing basketball, when syncope occured.Episode lasted for one to two minute, than patient woke on his own.
His mother also noticed that he has been having hearing problem.Physical Examination is normal.ECG showed prolonged QT interval with QTc of .49 second.What is most appropriate managment.

a. alpha adrenergic blocker
b. ACE inhibitor
c.Beta adrenergic blocker
d. Calcium channel blocker
e. Diuretic


The condition is Jervill and lange-Nielson syndrome
it is an autosomal recessive condition.
c/f..congenital deafness,recurrent syncope and sudden death due to v.tach,v.fib an dtorsades de pointes.
arrythmias are often triggered by stress and exercise.
treatment-beta blockers,k supplements and implantable defibrillators.

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