K76.81
BillableHepatopulmonary syndrome
HCC Category Mapping
V28HCC 63 — End-Stage Liver Disease
0.307V24HCC 27 — End-Stage Liver Disease
0.910ESRDHCC 27 — End-Stage Liver Disease
0.000What This Code Means
A condition where chronic liver disease causes abnormal blood vessel development in the lungs, leading to breathing difficulties and low oxygen levels.
Coding Tips
- •Code the underlying liver disease separately
- •Document pulmonary hypertension and oxygen requirements if present
Clinical Significance
Hepatopulmonary syndrome involves abnormal pulmonary vascular dilatation in patients with liver disease, causing severe hypoxemia and breathlessness. This rare but serious complication significantly impacts quality of life and may be an indication for liver transplantation.
Documentation Requirements
- ✓Chronic liver disease with portal hypertension
- ✓Intrapulmonary vascular dilatations on contrast echocardiography
- ✓Arterial oxygenation defect (A-a gradient >15 mmHg or >20 mmHg if >64 years)
- ✓Dyspnea and hypoxemia, especially when upright (orthodeoxia)
- ✓Positive bubble study or pulmonary angiography
- ✓Exclusion of primary cardiopulmonary disease
- ✓Digital clubbing may be present
- ✓Documentation of platypnea-orthodeoxia if present
Code First
Commonly Confused Codes
J44.1 — COPD with exacerbation when primary lung disease is presentI27.89 — Other specified pulmonary heart diseasesK76.6 — Portal hypertension underlying condition but HPS is the complicationR06.03 — Acute respiratory distress is symptom not diagnosisI26.99 — Pulmonary embolism when clot is suspected
Code Hierarchy
└K76Other diseases of liver└K76.8Other specified diseases of liver└K76.81Hepatopulmonary syndrome
└K76.81Hepatopulmonary syndrome