Hepatopulmonary syndrome
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Synopsis

Clinical manifestations include dyspnea, platypnea (dyspnea worsened by moving to upright position and relieved by lying flat), orthodeoxia (decrease in arterial oxygen by at least 5% when moving from supine to upright position), and nail clubbing. Patients will also have manifestations of chronic liver disease and portal hypertension (ie, spider angiomata, ascites, lower extremity edema, jaundice, asterixis, and encephalopathy).
HPS is a progressive disease, and mortality is typically linked to complications from underlying liver disease and not from hypoxic respiratory failure.
Codes
ICD10CM:K76.81 – Hepatopulmonary syndrome
SNOMEDCT:
371067004 – Hepatopulmonary Syndrome
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Last Reviewed:03/28/2018
Last Updated:03/18/2020
Last Updated:03/18/2020