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Hepatopulmonary syndrome

INTRODUCTION

Hepatopulmonary syndrome (HPS) is considered to be present in patients with the following triad [1-4]:

  • Liver disease
  • Increased alveolar-arterial gradient while breathing room air
  • Evidence for intrapulmonary vascular abnormalities, referred to as intrapulmonary vascular dilatations (IPVDs)

Estimates of the prevalence of HPS among patients with chronic liver disease range from 4 to 47 percent, depending upon the diagnostic criteria and methods used [2,5-9]. Mild hypoxemia is common among cirrhotic patients, and can result from the compression of lung parenchyma by ascites or pleural fluid [10]. Severe hypoxemia (PaO2 <60 mmHg) is less common and, in the absence of associated cardiopulmonary disease, should strongly suggest HPS [11,12].

The major clinical issues related to hepatopulmonary syndrome will be reviewed here. Portopulmonary hypertension and hepatic hydrothorax are also causes of dyspnea and hypoxemia among individuals with liver disease, and are discussed separately [1,3,13]. (See "Portopulmonary hypertension" and "Hepatic hydrothorax".)

CLINICAL MANIFESTATIONS

The clinical features of hepatopulmonary syndrome (HPS) are a combination of signs and symptoms that reflect hepatic and pulmonary dysfunction. More than 80 percent of patients present with symptoms of chronic liver disease; the remainder experience dyspnea as an initial symptom [14,15].
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