Use of L-Arginine for Inhaled Nitric Oxide (iNO) Dependent Hepatopulmonary Syndrome (HPS) Post-Liver Transplant

ABSTRACT

Hepatopulmonary syndrome (HPS) is one of the recognised complications of liver cirrhosis which warrants urgent liver transplantation. The symptoms usually improve post transplantation but might take 6-12 months to resolve completely. Severe hypoxemia post liver transplantation, especially when done for hepatopulmonary syndrome has been reported. iNO use for the same has been reported in literature with good response. However, it is practically very difficult to continue iNO in a stable child on a portable ventilator due to problems of scavenging.We report a case of 7-year-old girl who underwent orthotopic liver transplantation (OLT) for severe HPS. Post transplantation the patient had a stormy respiratory course and became severely hypoxemic. She was started on inhaled NO and showed a good response. However, the patient then became NO dependent and multiple attempts to wean iNO failed albeit minimal ventilatory requirements. Following IV L-Arginine infusion, iNO could be weaned within 6 hours without recurrence of hypoxemia. L-Arginine infusion should be considered as a treatment option when facing difficulties to wean iNO in an otherwise well responding patient.