The online version of this article (doi:10.1186/1752-1947-8-418) contains supplementary material, which is available to authorized users.
The authors declare that they have no competing interests.
MG reviewed the clinical course and participated in drafting and revising the manuscript. KWK participated in the literature review and in drafting and revising the manuscript. MGM provided radiological images and participated in drafting and revising the manuscript. TPS supervised the overall effort and participated in drafting and revising the manuscript. All authors read and approved the final manuscript.
Hepatopulmonary syndrome is a clinical syndrome that can affect patients of all ages with liver disease and is more common in children with biliary atresia. Contrast echocardiography is the test of choice to diagnose the presence of intrapulmonary vascular dilatation. The established treatment for hepatopulmonary syndrome is liver transplantation.
We present the case of an 8-month-old Caucasian baby boy with a history of biliary atresia, polysplenia, and interrupted inferior vena cava who presented with hypoxemia and cyanosis that progressed rapidly. A chest computed tomography angiogram revealed significant dilatation of the pulmonary vasculature, prompting further evaluation and diagnosis of hepatopulmonary syndrome with contrast echocardiography. He was maintained on a milrinone infusion while awaiting liver transplantation. His hypoxemia improved slowly following liver transplantation, requiring tracheostomy and prolonged ventilator dependence.
Hepatopulmonary syndrome should be included in the differential for progressive hypoxemia in children with liver disease, particularly those with biliary atresia. Imaging with chest computed tomography angiogram and contrast echocardiography should be considered in cases of unexplained refractory hypoxemia.
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Krowka MJ, Wiseman GA, Burnett OL, Spivey JR, Therneau T, Porayko MK, Wiesner RH: Hepatopulmonary syndrome: a prospective study of relationships between severity of liver disease, PaO 2 response to 100% oxygen, and brain uptake after (99m)Tc MAA lung scanning. Chest. 2000, 118 (3): 615-624. 10.1378/chest.118.3.615. CrossRefPubMed
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