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Journal of Medical Case Reports

, 8:418

First Online: 10 December 2014Received: 05 August 2014Accepted: 17 October 2014DOI: 10.1186-1752-1947-8-418

Cite this article as: Govindan, M.L., Kuo, K.W., Mahani, M.G. et al. J Med Case Reports 2014 8: 418. doi:10.1186-1752-1947-8-418


IntroductionHepatopulmonary 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.

Case presentationWe 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.

ConclusionsHepatopulmonary 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.

KeywordsContrast echocardiography Hepatopulmonary syndrome Pediatric Refractory hypoxemia AbbreviationsBNPBrain natriuretic peptide

CTComputed tomography

FiO2Fraction of inspired oxygen

HPSHepatopulmonary syndrome

IPVDIntrapulmonary vascular dilatation

MRIMagnetic resonance imaging

OLTOrthotopic liver transplantation

PaO2Partial pressure of oxygen in arterial blood

PICUPediatric intensive care unit


UNOSUnited Network for Organ Sharing.

Electronic supplementary materialThe online version of this article doi:10.1186-1752-1947-8-418 contains supplementary material, which is available to authorized users.

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Autor: Morgen L Govindan - Kevin W Kuo - Maryam Ghadimi Mahani - Thomas P Shanley


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