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• W4387249691 abstract "SESSION TITLE: Tissue Is the Issue SESSION TYPE: Rapid Fire Case Reports PRESENTED ON: 10/11/2023 12:00 pm - 12:45 pm INTRODUCTION: Hepatopulmonary syndrome (HPS) is a condition that leads to hypoxia due to chronic liver-related injury. The prevalence of HPS in cirrhosis is approximately 4-19% (1). HPS is a triad of liver disease, pulmonary gas exchange abnormalities, and widespread pulmonary vascular dilation (1). The pathophysiology leading to hypoxia is not definitively established, however, theories suggest mediators not cleared by the liver lead to pulmonary vasodilation affecting gas exchange. In this case, we present a gentleman with significant alcohol-related cirrhosis with asymptomatic hypoxia. CASE PRESENTATION: Patient is a 66-year-old male who presents to the pulmonary clinic with a chief complaint of dyspnea on exertion. In clinic he was asymptomatic with a resting room air oxygen saturation (SPO2) of 87% on room air. His diagnosis of cirrhosis was made several years ago following an upper gastrointestinal bleed that led to an esophagogastroduodenoscopy (EGD). Portal hypertension was noted on EGD and cirrhosis with splenomegaly were documented on imaging. The patient had a MELD score that went from 14 to 11 after alcohol cessation. Work up for the hypoxia included an ABG (pH of 7.41, CO2 27, O2 57 on room air) with a P/F of 271 and A-a gradient of 59. Spirometry was normal but DLCO and KCO were severely reduced. An echocardiogram showed an ejection fraction of 55-60% with grade 1 diastolic dysfunction. A bubble study was positive for a large right-to-left shunt. A trans-esophageal echocardiogram confirmed that the shunt originated in the pulmonary vasculature, and a CT of the chest showed dilated pulmonary vasculature which confirmed the diagnosis. He was started on supplemental oxygen and his transplant status was updated appropriately. DISCUSSION: HPS was first described in 1977 as a condition that involved hypoxemia, hypocapnia, and orthodeoxia with normal spirometry (3). It is present in anywhere from 4-19% of cirrhotics and does not necessarily correlate with the severity of liver disease or portal hypertension. In cirrhotics not eligible for transplant, the 5-year survival is 23% vs 63% for those who do and do not have HPS, respectively. However, patients with HPS do as well after liver transplant as those without this condition. Therefore, the presence of HPS requires higher priority for transplant for those who are eligible. Beyond transplant there are no therapies with proven benefit. Supplemental oxygen is prescribed for all patients with HPS who qualify. CONCLUSIONS: In summary – HPS is a condition that develops in patients with cirrhosis. It has a high mortality rate and doesn't correlate well with the severity of liver disease. Despite the absence of available treatment options its identification is important for prioritizing the need for transplant. REFERENCE #1: Schenk P, Fuhrmann V, Madl C, Funk G, Lehr S, Kandel O, Müller C. Hepatopulmonary syndrome: prevalence and predictive value of various cut offs for arterial oxygenation and their clinical consequences. Gut. 2002 Dec;51(6):853-9. doi: 10.1136/gut.51.6.853. PMID: 12427789; PMCID: PMC1773478. REFERENCE #2: Kennedy TC, Knudson RJ. Exercise-aggravated hypoxemia and orthodeoxia in cirrhosis. Chest. 1977 Sep;72(3):305-9. doi: 10.1378/chest.72.3.305. PMID: 891282. REFERENCE #3: Hepatopulmonary syndrome — a liver-induced lung vascular disorder | nejm. (n.d.). Retrieved March 31, 2023, from https://www.nejm.org/doi/full/10.1056/NEJMra0707185 DISCLOSURES: My spouse/partner as a Employee relationship with United Health Group Please note: $20001 - $100000 by Aaron Holley, value=stock options Write pieces on journal articles to be published online relationship with WebMD Please note: 5 years by Aaron Holley, value=Consulting fee Designed educational material relationship with CHEST Please note: 2014-2022 by Aaron Holley, value=Honoraria<br Consultant relationship with Metapharm Please note: 2022 by Aaron Holley, value=Consulting fee No relevant relationships by Imran Khan" @default.
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• W4387249691 title "HEPATOPULMONARY SYNDROME: ASYMPTOMATIC HYPOXIA IN THE OUTPATIENT SETTING" @default.
• W4387249691 doi "https://doi.org/10.1016/j.chest.2023.07.3136" @default.
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