Hepatopulmonary syndrome

This code identifies a complex pulmonary complication of liver disease, characterized by impaired arterial oxygenation due to intrapulmonary vascular dilatations. It results from chronic liver dysfunction leading to a ventilation-perfusion mismatch and hypoxemia.

Assign this code for patients presenting with liver cirrhosis or other chronic liver diseases who develop dyspnea, hypoxemia, and evidence of intrapulmonary shunting. Documentation should clearly indicate the presence of both liver disease and respiratory compromise consistent with hepatopulmonary syndrome, often confirmed by contrast-enhanced echocardiography or macroaggregated albumin lung perfusion scan.

• Ensure documentation specifies the underlying liver disease, as this is a secondary complication.
• Do not confuse with portopulmonary hypertension, which involves pulmonary arterial hypertension rather than intrapulmonary shunting.
• Code any associated symptoms or underlying liver conditions separately to provide a complete clinical picture.