The development of intrapulmonary arteriovenous shunts as a poor prognostic factor following surgery for biliary atresia.

ABSTRACT

Background & Aims: Chronic liver disease requiring liver transplantation is a common occurrence following corrective surgery for extrahepatic biliary atresia (EHBA). The formation of intrapulmonary arteriovenous shunts (IPS) is a well-known feature of chronic liver disease. The aim of this study was to investigate the development of IPS and its prognostic significance in postoperative patients with EHBA. Methods: Fourteen patients who underwent Kasai’s portoenterostomy during 1993-2005 were included in the study. The clinical features, hepatobiliary scintigraphy and biochemical liver function tests were recorded. A transthoracic contrast enhanced echocardiogram using a four-chamber view was performed in all patients within a week of the Kasai’s procedure by injecting 5 mL of hand-agitated saline solution into a peripheral vein. The opacification of microbubbles in the left atrium 3-6 minutes after their emergence in the right atrium was considered diagnostic of IPS. The contrast enhanced echocardiogram was repeated 6 months after the Kasai’s procedure in all patients Results: Nine patients were clinically asymptomatic after surgery. HIDA scan was excretory in all 14 patients at the time of the study, although 5 patients were jaundiced. The serum bilirubin increased in 2 patients after surgery; both these patients were jaundiced and developed ascites and 1 expired one year after surgery. Contrast enhanced echocardiogram was negative for IPS in all 14 initially. In the follow-up evaluation the only patient who died was the one who had developed IPS. Conclusions: There is a risk of developing IPS following a failed Kasai’s portoenterostomy in patients of EHBA. Contrast enhanced echocardiography can be used to serially monitor these patients for early detection of this complication. It can be used to predict prognosis after hepatic portoenterostomy and can be extremely useful in selecting patients who will need liver transplant on priority.