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A6764 - Hepatopulmonary Syndrome Masquerading as Interstitial Lung Disease
Author Block: C. C. Odigwe1, L. B. Tlhabano2, R. M. Gripaldo3; 1Division of Pulmonary, Critical Care and Sleep Medicine, Palmetto Health/University of South Carolina School of Medicine, Columbia, SC, United States, 2Division of Pulmonary, Critical Care and Sleep Medicine, Palmetto Health/USC School of Medicine, Columbia, SC, United States, 3Department of Medicine, University of South Carolina, Columbia, SC, United States.
The hepatopulmonary syndrome is a well described entity in the setting of chronic liver disease. When present it portends a poor prognosis and warrants referral for liver transplantation. Here we present a patient that was referred to our Interstitial Lung Disease (ILD) Clinic for hypoxemia and an abnormal chest computed tomography (CT). A 64-year-old woman was seen in the pulmonary clinic for the evaluation of hypoxemia and non specific interstitial changes on recent chest CT imaging. Hypoxemia had been noted during a recent hospitalization for presumed Wernicke’s encephalopathy. Preliminary evaluation was suspicious for possible ILD so she was referred to the ILD Clinic. She gave a history of dyspnea over the past six to eight months. She reported improved dyspnea and pulse oximetry when she was recumbent. She was noted to have finger clubbing and spider angiomas. She was severely hypoxemic and required 8 liters of supplemental oxygen during her walk test. She underwent an urgent CT pulmonary angiogram (CTA) to exclude a pulmonary embolism. There was no pulmonary embolism but the CTA showed dilated pulmonary vasculature extending out to the periphery. She did have basal sub-pleural reticular changes in her lung parenchyma but disproportionate to her degree of hypoxemia. Echocardiogram with bubble study showed large bubbles in the left atrium noted on beats six and seven indicating an extra-cardiac shunt. She had normal left and right ventricular functions. She was then referred up for liver transplantation. Hepatopulmonary syndrome (HPS) is characterized by the triad of abnormal arterial oxygenation caused by intrapulmonary vascular dilatations in the setting of liver disease, portal hypertension, or congenital portosystemic shunts. The pathogenesis may involve increased bacterial translocation and toxin release (intestinal endotoxemia) from portal hypertension stimulating the release of vasoactive mediators, failure of the damaged liver to clear circulating pulmonary vasodilators, production of circulating vasodilators by the damaged liver, and inhibition of circulating vasoconstrictive substances by the damaged liver. Our patient presented with dyspnea which is seen in over 80% of patients and she also had spider angiomas. The presence of cutaneous spider angiomas is associated with more systemic and pulmonary vasodilation and associated with HPS. She was referred to the ILD clinic due to hypoxemia and an abnormal chest CT however her real problem was HPS. This case highlights the importance of a thorough clinical history and putting key clinical findings in the context of the overall multisystem presentation.