Development of intrapulmonary arteriovenous shunting in postoperative biliary atresia: evaluation by contrast-enhanced echocardiography.

This report presents biliary atresia (BA) patients with intrapulmonary arteriovenous shunting (IPS), which was evaluated suitably by contrast-enhanced echocardiography (CEC). Of 88 BA patients seen in the last 20 years, 8 (9.1%) had IPS at 8 months to 16 years of age. Two were associated with polysplenia syndrome, 1 had persistent jaundice after hepatic portoenterostomy, and 2 underwent splenorenal shunt. According to the comparison between microbubbles in left atrium (LA) and in right atrium (RA) detected by CEC, IPS was classified as grade I, mild (LA < RA); grade II, moderate (LA < RA); grade II, severe (LA = RA). Grade I consisted of 4 patients whereas grade II and III held 2 patients each. Clinical symptoms such as cyanosis, exertional dyspnea, or clubbing were present in 50% of grade I and all of grade II and IlI. Mean PaO2 in grade I, II, and III was 70.5, 50.4, and 35.3 mm Hg, respectively. In 1 patient with grade I, IPS spontaneously disappeared, but pulmonary hypertension developed later. One patient in grade II died of pulmonary complications, and the other is considered a candidate for livertransplantation (LTx). One patient in grade III died of liver failure, whereas the other is free of IPS after LTx. IPS can lead to a life-threatening complication in postoperative BA patients, and CEC may be a convenient and useful method to evaluate the degree of IPS and determine therapeutic strategy.