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Hepatopulmonary Syndrome: The Case of the Misleadi ...
Hepatopulmonary Syndrome: The Case of the Misleading Shunt
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This case report describes a 60-year-old man with cirrhosis from metabolic and alcohol-associated liver disease who was found incidentally to have severe hypoxemia after a fall. Although he had no dyspnea, his oxygen saturation was 70% on room air and worsened when supine. Exam showed clubbing and cyanosis, and his arterial blood gas demonstrated hypoxemia with an elevated A–a gradient.<br /><br />Initial transthoracic echocardiography with agitated saline suggested an intracardiac shunt because bubbles appeared in the left atrium early, raising concern for an atrial septal defect or patent foramen ovale. However, further evaluation with right heart catheterization and intracardiac echo found no intracardiac shunt. A 99mTc-MAA perfusion lung scan instead showed extrapulmonary activity, confirming a right-to-left shunt from the lungs. The final diagnosis was hepatopulmonary syndrome (HPS), a complication of chronic liver disease caused by intrapulmonary vascular dilatations.<br /><br />The discussion emphasizes that HPS is a potentially reversible but high-mortality cause of hypoxemia, and liver transplantation is the definitive treatment. Importantly, the case highlights a diagnostic pitfall: in severe HPS, saline contrast echocardiography can show early bubble transit that mimics an intracardiac shunt. Therefore, clinicians should not stop the workup at a presumed ASD/PFO if the clinical picture still suggests HPS.<br /><br />The authors conclude that hospitalists play a key role in pursuing definitive testing when initial studies are misleading, to avoid missing serious but treatable disease.
Asset Subtitle
Jessica M. Guynes
Meta Tag
Author List
Akriti Jalla, Erika Abel, Jessica M. Guynes, Miranda Sullivan
Category
Clinical Vignettes
Concept
Hepatopulmonary Syndrome
Concept
Hypoxemia
Concept
Intrapulmonary Vascular Dilatation
Concept
Liver Transplantation
Concept
Chronic Liver Disease
Distinguished
Non-Finalist
Presenter Organization
University of South Florida Morsani College of Medicine
Presenting Author
Jessica M. Guynes
Track
Adult
Keywords
hepatopulmonary syndrome
cirrhosis
hypoxemia
right-to-left shunt
intrapulmonary vascular dilatation
saline contrast echocardiography
intracardiac shunt
99mTc-MAA perfusion scan
liver transplantation
chronic liver disease
Hepatopulmonary Syndrome
Hypoxemia
Intrapulmonary Vascular Dilatation
Liver Transplantation
Chronic Liver Disease
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