Clinical significance of hepatopulmonary syndrome in biliary atresia

Abstract

(Objectives)The clinical characteristics of intrapulmonary shunt (IPS) that can lead to development of hepatopulmonary syndrome (HPS) in biliary atresia (BA) have not been well described. Therefore, we investigated the incidence and clinical significance of IPS and HPS in BA.

(Methods)We evaluated 72 patients with BA between March 2010 and May 2013 for diagnosis of IPS by contrast-enhanced echocardiography (CEE) and HPS by arterial blood-gas analysis (ABGA). ABGA was performed only in patients who had IPS by CEE. Clinical data were reviewed retrospectively by grouping patients; normal group in which patients had no evidence of IPS by CEE, IPS group in which patients had IPS but did not have any evidence of HPS by ABGA, and HPS group in which patients had evidences of both of IPS and HPS.

(Results)IPS was identified in 41 patients (56.9%) and normal group was identified in 31 patients (43.1%). Six patients with IPS were excluded from data analysis because ABGA were not examined in these patients. IPS group included 20 patients, and HPS group included 15 patients. Compared to the normal group, the bilirubin level at the time of CEE and the hepatic

fibrosis score were significantly increased in the IPS group and HPS group, and liver transplantation was performed significantly more frequent in the HPS group.

(Conclusion)The incidence of IPS in BA was considerably high. Patients with IPS have worse clinical outcomes than patients without IPS. The identification and close-monitoring of IPS in BA lead to favorable outcomes by preventing the late liver transplantation.