Anesthetic management of a pediatric patient with pulmonary arteriovenous fistula undergoing liver transplantation - a case report.

For patients with HPS who require anesthesia for a procedure, HPV should be maintained to prevent worsening hypoxemia. Here, the case of a 9-yr-old girl who was scheduled for a living donor liver transplantation is presented. The patient suffered from end-stage liver disease with HPS due to biliary atresia, which contributed to the development of a diffuse pulmonary AVF. Consequently, anesthetic management of this patient involved two different types of pulmonary shunt. It is important to maintain HPV, not only to prevent worsening of the hypoxia caused by HPS but also to inhibit an increase in PVR that could cause an increase of shunt flow through the pathological fistula. A TIVA technique was performed, and a nitrous oxide inhaler was prepared in case of a possible increase in PVR during the reperfusion period. There were no adverse events during the operation. Thus, anesthesiologists should be aware of the pathophysiological status of HPS and its potential to progress to a pulmonary AVF in order to meticulously determine an anesthesia plan that accounts for the hypoxia and PVR that are associated with HPS.