The development of intrapulmonary arteriovenous shunts as a poor prognostic factor following surgery for biliary atresia.
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Date
2009-04
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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.
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extrahepatic biliary atresia, chronic liver disease, intra-pulmonary arteriovenous shunt, liver transplantation
Citation
Agarwal G S, Saxena A, Bhatnagar V. The development of intrapulmonary arteriovenous shunts as a poor prognostic factor following surgery for biliary atresia. Tropical Gastroenterology. 2009 Apr-June; 30(2): 110-112.