Systemic paediatrics: congenital long QT syndrome: practice question

A Pediatrics question in the style of the NEET PG paper. Answer it first, then read why the right option is right and why each of the others is wrong.

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Pediatrics · Systemic paediatrics: congenital long QT syndrome

Sample item

A 6-year-old deaf child has had syncope during a swimming race and after a sudden loud noise. ECG shows a corrected QT interval of 520 ms. Which drug is the mainstay of treatment?

AAmiodaroneBA beta blocker such as propranololCDigoxinDSotalol

Original practice item, not a recalled exam question. Nothing here is recorded.

The answer and the reasoning

B. A beta blocker such as propranolol

Deafness with syncope and a long QT interval is the Jervell and Lange-Nielsen form of congenital long QT syndrome, which is autosomal recessive. Beta blockers reduce adrenergic triggers of torsades de pointes and are first-line, with an ICD for high-risk cases. Drugs that prolong the QT interval must be avoided.

Option by option

A. Amiodarone
Amiodarone prolongs the QT interval and can provoke torsades de pointes.
B. A beta blocker such as propranolol (correct)
Correct. Beta blockade reduces sympathetic triggers and the risk of fatal arrhythmia.
C. Digoxin
Digoxin does not control the arrhythmia and is not indicated.
D. Sotalol
Sotalol prolongs repolarisation and is contraindicated.

Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.

Questions about this Pediatrics question

What is the answer to this Pediatrics question?

The best answer is B, A beta blocker such as propranolol. Deafness with syncope and a long QT interval is the Jervell and Lange-Nielsen form of congenital long QT syndrome, which is autosomal recessive.

Why is "Amiodarone" not the answer?

Amiodarone prolongs the QT interval and can provoke torsades de pointes.

Why is "Digoxin" not the answer?

Digoxin does not control the arrhythmia and is not indicated.

Why is "Sotalol" not the answer?

Sotalol prolongs repolarisation and is contraindicated.

What concept does this question test?

It tests Systemic paediatrics: congenital long QT syndrome in Pediatrics. Beta blockers reduce adrenergic triggers of torsades de pointes and are first-line, with an ICD for high-risk cases. Drugs that prolong the QT interval must be avoided.

How hard is this question?

We tag it as an application question: it asks you to use a fact on a patient or a finding. The tag is our own editorial judgement, not an official difficulty rating, so read the reason for every option, not only the answer.

How should I study Systemic paediatrics: congenital long QT syndrome?

Memorise milestones and the vaccine schedule as tables and revise them often. Then use the flashcards and the other Pediatrics questions on this site to test what stuck.

Is this a real NEET PG question?

No. It is an original practice item (editorial status: sample, version 1). NextPrep does not publish recalled or leaked questions, and independent faculty review of this item has not been completed yet, so confirm important facts in a standard textbook.