S5711 Hepatopulmonary Syndrome: A Case of Severe Pulmonary Vascular Dilatation in Newly Diagnosed Sarcoid Cirrhosis

Introduction: Hepatopulmonary syndrome (HPS) is a well-described, but significant complication of cirrhosis. It is relatively uncommon with a 10-30% occurrence rate. Cirrhosis is a rare manifestation of sarcoidosis. Here we describe a case of severe HPS in a patient with known sarcoidosis and newly diagnosed cirrhosis. Case Description/Methods: A 63-year-old woman with a history of tobacco use and sarcoidosis, diagnosed by liver biopsy 20 years prior, presented with chronic, worsening hypoxia and dyspnea on exertion. She had finger clubbing and an oxygen saturation of 70%, requiring 15 L of high flow nasal cannula. Labs were notable for polycythemia with a hemoglobin of 21 and hypoxemia with pH 7.370, partial pressure of carbon dioxide 38, and partial pressure of oxygen (PaO2) 45. Chest computed tomography (CT) demonstrated interstitial fibrosis and dilatation of pulmonary vasculature in bilateral lower lung zones. CT angiography showed numerous dilated venous structures in bilateral lower lobes, suggestive of varices, and hepatic steatosis. Transthoracic echocardiogram showed extra-cardiac shunting without pulmonary hypertension. Liver ultrasound showed newly discovered nodular morphology consistent with cirrhosis, and cardiac positron emission tomography was negative for cardiac sarcoidosis. Upon discussion with Lung Transplant, the degree of hypoxemia was determined to not be consistent with sarcoid-associated interstitial lung disease. Instead, findings were thought to be due to HPS given her newly diagnosed cirrhosis, extra-cardiac shunting, and persistent PaO2 of less than 50 on room air. Cirrhosis was thought to be of sarcoid, with possible metabolic dysfunction-associated steatotic liver disease, etiology. Liver transplant was deferred at that time based on questionable benefit, high risk, and a stable Model for End-Stage Liver Disease score of 12. Discussion: HPS severity is determined by arterial oxygen content and the difference in alveolar and arterial oxygen content. Criteria for the most severe form of HPS includes a PaO2 less than 50 and an alveolar-arterial oxygen gradient greater than 15 on room air, as demonstrated by this patient. However, profound hypoxemia in HPS is uncommon and tends to be seen in more advanced cases. Due to the rarity of significant hepatic manifestations in sarcoidosis, HPS is an underrecognized complication of chronic liver disease in this setting. Further understanding is needed to better classify the differences of HPS from that of other causes.