A 48-year-old man with Child-Pugh C cirrhosis reports breathlessness relieved on lying flat and worsened on standing upright. Room air SpO2 is 89 percent sitting and drops to 84 percent standing. Transthoracic contrast echocardiography shows delayed appearance of microbubbles in the left atrium after four cardiac cycles. What is the diagnosis?

• A Portopulmonary hypertension
• B Hepatopulmonary syndrome ✓
• C Hepatic hydrothorax with shunt
• D Pulmonary embolism

Explanation

The triad of liver disease, arterial deoxygenation, and intrapulmonary vascular dilatation defines hepatopulmonary syndrome. Dilated precapillary vessels create shunts that delay bubble transit, so contrast reaches the left heart after several beats rather than instantly as in an atrial septal defect. Orthodeoxia arises because basal dilation worsens in the upright position, worsening ventilation-perfusion mismatch. Portopulmonary hypertension instead shows elevated pulmonary arterial pressure on echocardiography.

Reference: Harrison's Principles of Internal Medicine, 21st ed.

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Written and medically reviewed by the StethoPrep medical team.