Hepatopulmonary syndrome

This code represents hepatopulmonary syndrome (HPS), a serious complication of liver disease characterized by a triad of chronic liver disease, intrapulmonary vascular dilatations, and impaired arterial oxygenation. It results from abnormal pulmonary vascular responses to liver dysfunction, leading to hypoxemia.

Assign this code when documentation clearly indicates a diagnosis of hepatopulmonary syndrome in a patient with underlying liver disease. Supporting documentation typically includes evidence of hypoxemia, intrapulmonary shunting (e.g., contrast echocardiography, macroaggregated albumin scan), and clinical signs of liver disease.

• Ensure the documentation explicitly states "hepatopulmonary syndrome" rather than just general pulmonary complications of liver disease.
• Always code the underlying liver disease first, as HPS is a complication of liver dysfunction.
• Do not confuse with portopulmonary hypertension (I27.21), which involves pulmonary arterial hypertension, not intrapulmonary vascular dilatations.