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Hepatopulmonary syndrome in adults: Prevalence, causes, clinical manifestations, and diagnosis

Paul A Lange, MD
James K Stoller, MD, MS
Section Editor
Jess Mandel, MD
Deputy Editor
Geraldine Finlay, MD


Hepatopulmonary syndrome (HPS) is characterized by the triad of abnormal arterial oxygenation caused by intrapulmonary vascular dilatations (IPVDs) in the setting of liver disease, portal hypertension, or congenital portosystemic shunts [1].

The prevalence, causes, clinical manifestations, and diagnostic evaluation of HPS are reviewed here. The natural history, treatment, and outcomes of HPS are discussed separately. (See "Hepatopulmonary syndrome in adults: Natural history, treatment, and outcomes".)


Estimates of the prevalence of HPS among patients with chronic liver disease range from 4 to 47 percent (on average one quarter), depending upon the diagnostic criteria, methods used, and population studied [2-13]. As examples:

In one prospective study of 111 patients with cirrhosis, HPS was reported in 24 percent when transthoracic contrast echocardiography, blood gas analysis, and pulmonary function testing were used for its detection [2].

In another prospective study of patients with liver cirrhosis, 26 percent had HPS by similar criteria [7].

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Literature review current through: Nov 2017. | This topic last updated: Dec 21, 2016.
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