A 50-year-old woman with cirrhosis presents with progressive dyspnea and cyanosis. Physical examination reveals clubbing and spider nevi. Room air blood gas shows PaO2 of 55 mmHg. Contrast-enhanced echocardiography shows delayed appearance of microbubbles in the left atrium after 3-6 cardiac cycles. Which is the definitive treatment?

• A Liver transplantation ✓
• B Transjugular intrahepatic portosystemic shunt (TIPS)
• C Supplemental oxygen
• D Pentoxifylline

Explanation

Hepatopulmonary syndrome is defined by the triad of liver disease, intrapulmonary vascular dilatations (shown by delayed bubbles on contrast echo after 3-6 cycles), and hypoxemia (PaO2 less than 70 mmHg). Liver transplantation is the only definitive treatment, with most patients showing improvement or resolution of hypoxemia post-transplant. TIPS does not improve oxygenation in this condition.

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

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Written and medically reviewed by the StethoPrep medical team.