Het hepatopulmonaal syndroom

Background

Hepatopulmonary syndrome is a severe complication of liver disease, with greatly increased mortality. The syndrome is characterized by increased blood-flow, intrapulmonary vasodilatation and angiogenesis, leading to effects including the formation of shunts. This leads to a decrease in arterial oxygen pressure. Liver transplantation is the only effective treatment.

Case description

A 74-year-old woman with cirrhosis of the liver attended the pulmonary outpatients’ clinic with progressive dyspnoea, which worsened if she sat upright from a lying position (platypnoea). Contrast echocardiography confirmed the diagnosis ‘hepatopulmonary syndrome’. The patient was not eligible for liver transplantation. She was given oxygen therapy and died from decompensated cirrhosis of the liver eighteen months later.

Conclusion

Early recognition of hepatopulmonary syndrome is important, because patients may be given priority for liver transplantation. Contrast echocardiography is indicated in patients with liver disease and suffering from hypoxaemia for which there is no other explanation, to reveal the presence of intrapulmonary shunt.

Conflict of interest and financial support: none declared.