Hepatology: hepatopulmonary syndrome: 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 · Hepatology: hepatopulmonary syndrome

Sample item

A 55-year-old man with cirrhosis has progressive breathlessness that is worse when sitting upright and better when lying flat. Oxygen saturation is 88% sitting and 95% supine. He has clubbing and spider naevi, and the chest radiograph and spirometry are normal. Which test best confirms the underlying mechanism?

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

The answer and the reasoning

A. Contrast (bubble) echocardiography showing delayed microbubbles in the left atrium

Hepatopulmonary syndrome is intrapulmonary vascular dilatation in liver disease, causing hypoxaemia that worsens upright (platypnoea-orthodeoxia) because the dilated vessels lie in the lung bases. Agitated saline contrast echocardiography shows microbubbles reaching the left atrium after three to six cardiac cycles, unlike an intracardiac shunt in which they appear within three beats. Liver transplantation is the definitive treatment.

Option by option

  • A. Contrast (bubble) echocardiography showing delayed microbubbles in the left atrium (correct)

    Late appearance of bubbles in the left atrium demonstrates intrapulmonary shunting through dilated vessels.

  • B. High-resolution CT chest for interstitial fibrosis

    Interstitial disease causes restrictive physiology and a reticular pattern, which are absent here.

  • C. CT pulmonary angiography to look for emboli

    Embolism is unlikely with a normal radiograph and positional hypoxaemia, and CT angiography does not demonstrate dilated capillaries.

  • D. Right heart catheterisation showing raised mean pulmonary artery pressure

    Raised pulmonary pressure suggests portopulmonary hypertension, which does not typically cause orthodeoxia.

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.

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Questions about this General Medicine question

What is the answer to this General Medicine question?

The best answer is A, Contrast (bubble) echocardiography showing delayed microbubbles in the left atrium. Hepatopulmonary syndrome is intrapulmonary vascular dilatation in liver disease, causing hypoxaemia that worsens upright (platypnoea-orthodeoxia) because the dilated vessels lie in the lung bases.

Why is "High-resolution CT chest for interstitial fibrosis" not the answer?

Interstitial disease causes restrictive physiology and a reticular pattern, which are absent here.

Why is "CT pulmonary angiography to look for emboli" not the answer?

Embolism is unlikely with a normal radiograph and positional hypoxaemia, and CT angiography does not demonstrate dilated capillaries.

Why is "Right heart catheterisation showing raised mean pulmonary artery pres…" not the answer?

Raised pulmonary pressure suggests portopulmonary hypertension, which does not typically cause orthodeoxia.

What concept does this question test?

It tests Hepatology: hepatopulmonary syndrome in General Medicine. Agitated saline contrast echocardiography shows microbubbles reaching the left atrium after three to six cardiac cycles, unlike an intracardiac shunt in which they appear within three beats. Liver transplantation is the definitive treatment.

How hard is this question?

We tag it as a multi-step question: it asks you to combine more than one idea. 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 Hepatology: hepatopulmonary syndrome?

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.