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Hepatopulmonary syndrome in adults: Natural history, treatment, and outcomes

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 (IPVD) in the setting of liver disease, portal hypertension, or congenital portosystemic shunts [1,2].

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


HPS is typically a progressive disorder, the presence of which worsens the prognosis of patients with cirrhosis and probably other liver diseases [1,3-7]. The cause of death among patients with HPS tends to be multifactorial and related to complications of underlying liver disease (eg, hepatic failure, multisystem organ failure due to sepsis, hepatocellular cancer, gastrointestinal bleeding) rather than from HPS-related hypoxemic respiratory failure [1,3,4]. Spontaneous resolution of HPS without treatment is unlikely. These conclusions are derived from the following studies:

One observational study of 138 liver transplant candidates reported a higher mortality in patients with HPS compared with case-matched controls (78 versus 43 percent) [4]. Similarly, patients with HPS had a lower median survival (24 versus 87 months) and lower five-year survival (23 versus 63 percent).

A single center prospective study of 316 patients referred for liver transplantation reported that compared with patients without HPS, patients with HPS had a nonsignificant increase in waitlist mortality (24 versus 16 percent) and a slightly lower pre-liver transplant survival (35 versus 42 months) [5].

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