Cardiology: Noonan syndrome cardiac lesion: practice question

A General Medicine 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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General Medicine · Cardiology: Noonan syndrome cardiac lesion

Sample item

A 6-year-old boy has short stature, a webbed neck, low-set ears, ptosis and pectus excavatum. An ejection systolic murmur is heard at the upper left sternal border, preceded by an ejection click. Which cardiac lesion is most characteristic of his syndrome?

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

The answer and the reasoning

B. Pulmonary valve stenosis

This phenotype with normal chromosomes in a boy is Noonan syndrome, in which pulmonary valve stenosis and hypertrophic cardiomyopathy are the typical cardiac lesions. Coarctation is the lesion of Turner syndrome and atrioventricular septal defect goes with Down syndrome.

Option by option

  • A. Atrioventricular septal defect

    Atrioventricular septal defect is the typical defect of Down syndrome.

  • B. Pulmonary valve stenosis (correct)

    Correct. Dysplastic pulmonary valve stenosis is the classic lesion, matching the click and murmur at the pulmonary area.

  • C. Aortic valve stenosis

    Aortic stenosis gives a murmur at the right upper sternal edge radiating to the neck, with no click at the pulmonary area.

  • D. Coarctation of the aorta

    Coarctation of the aorta is typical of Turner syndrome in girls.

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.

More General Medicine questions

Questions about this General Medicine question

What is the answer to this General Medicine question?

The best answer is B, Pulmonary valve stenosis. This phenotype with normal chromosomes in a boy is Noonan syndrome, in which pulmonary valve stenosis and hypertrophic cardiomyopathy are the typical cardiac lesions.

Why is "Atrioventricular septal defect" not the answer?

Atrioventricular septal defect is the typical defect of Down syndrome.

Why is "Aortic valve stenosis" not the answer?

Aortic stenosis gives a murmur at the right upper sternal edge radiating to the neck, with no click at the pulmonary area.

Why is "Coarctation of the aorta" not the answer?

Coarctation of the aorta is typical of Turner syndrome in girls.

What concept does this question test?

It tests Cardiology: Noonan syndrome cardiac lesion in General Medicine. Coarctation is the lesion of Turner syndrome and atrioventricular septal defect goes with Down syndrome.

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 Cardiology: Noonan syndrome cardiac lesion?

For each disease know the classic presentation, the best test, and the first-line treatment. Then use the flashcards and the other General Medicine 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.