RESUMO
BACKGROUND: The hepatopulmonary syndrome with profound hypoxemia is a rare but severe complication for children with liver cirrhosis. It can be reversed by liver transplantation (LT), which is now regarded as a good indication. However, previous reports have described cases of transient or fatal deteriorations of intrapulmonary shunting after pediatric liver transplantation with dramatically worsening hypoxemia. METHODS AND RESULTS: A similar case during and after LT in a 4-year-old girl with severe hepatopulmonary syndrome is described with prompt reversal of hypoxemia by inhaled nitric oxide, which was discontinued definitely until day 14 after LT. CONCLUSIONS: During or after LT, worsening hypoxemia may be improved by using inhaled nitric oxide in pediatric patients undergoing liver transplantation for liver cirrhosis and hepatopulmonary syndrome. The mechanisms are unclear, but may involve mismatching lung ventilation-perfusion. However, additional clinical reports are necessary before accepting these results.