S5381 Hepatopulmonary Syndrome Ought to Be Considered in Patients With Chronic Liver Disease and Persistent Hypoxia

Introduction: Hepatopulmonary syndrome (HPS) is characterized by the triad of abnormal arterial oxygenation caused by intrapulmonary vascular dilatations (IPVDs) in the setting of liver disease or portal hypertension. Transthoracic contrast echocardiogram (TTCE) with agitated saline bubble study should be considered for patients with persistent hypoxia and chronic liver disease. Case Description/Methods: A 69-year-old woman with metabolic dysfunction-associated steatohepatitis-related cirrhosis, splenic and esophageal varices, chronic pancytopenia, type 2 diabetes mellitus, hyperlipidemia, remote history of deep vein thrombosis presented with dyspnea on exertion and lightheadedness. Of note, the patient had 2 prior hospitalizations for atypical chest pain accompanied by shortness of breath in the span of 6 months. She underwent transthoracic contrast echocardiography to assess for intracardiac shunt that demonstrated positive bubble study with evidence of right-to-left shunt physiology, but undetermined if bubbles were early versus late in their timing of shunt. Transesophageal echocardiogram was deferred for concerns of esophageal varices. A second bubble study showed bubbles clearly entering the left atrial cavity through pulmonary veins after 3 cardiac cycles. Bubbles appeared not to cross the atrial wall. This is most consistent with intrapulmonary shunt and with conjunction of underlying cirrhosis HPS was diagnosed. At an initial evaluation with a hepatologist, arterial blood gas revealed PaO2 of 61 mm Hg on room air that corrected to 400 mm Hg with 100% FiO2. Repeat arterial blood gas test 2 months later showed partial pressure of oxygen 55 mm Hg on room air that improved to 348 mm Hg on 100% FiO2. She met the criteria for moderate to severe Type 1 HPS. She eventually underwent liver transplantation and is doing very well, on room air on follow up. Discussion: Most patients with HPS present with symptoms and signs of chronic liver disease, that are neither sensitive nor specific for HPS. Transthoracic contrast echocardiography with agitated saline is the gold standard for diagnosing pulmonary vascular dilatation. Based on observational studies, liver transplantation demonstrates complete or near complete resolution of HPS with improved oxygenation and shunt in the majority (about 80%) of patients within 6 to 12 months. Given the nature of rapid progression and poor prognosis, it is crucial to have a high suspicion for HPS in a patient with liver disease and hypoxia for prompt referral for liver transplant evaluation.