Updated: 10/30/2019

Hepatopulmonary Syndrome

Review Topic
  • A 60-year-old man presents with increasing shortness of breath. He reports that this symptom worsens when he is in the upright position and improves when he is laying in bed. Medical history is significant for end-stage liver disease due to hepatitis C infection. On physical exam, there is decreased breath sounds on pulmonary auscultation and spider nevi. Arterial blood gas analysis is significant for an alveolar-arterial gradient of 20 mmHg.
  • Clinical definition
    • liver disease leading to severe pulmonary vascular complications
  • Epidemiology
    • incidence
      • dependent on the patient population and diagnostic criteria
        • in patients with cirrhosis it is approximately 15-30%
    • demographic
      • the middle-aged
      • no sex difference
    • location
      • intrapulmonary vasculature
    • risk factors
      • liver disease
      • portal hypertension
        • can be with or without cirrhosis
  • Pathophysiology
    • pathoanatomy
      • unclear
      • believed to be due to increased vasodilator (e.g., nitric oxide and carbon monoxide) production secondary to liver disease resulting in
        • ventilation-perfusion (V/Q) mismatch
        • alveolar-capillary oxygen disequilibrium
  • Associated conditions
    • liver disease
    • increased alveolar-arterial (A-a) gradient 
  • Prognosis
    • liver transplantation may result in resolution of hepatopulmonary syndrome
  • Symptoms
    • dyspnea on exertion or at rest
    • platypnea
      • increased dyspnea when upright and improves when supine
    • weakness and fatigue
  • Physical exam
    • orthodeoxia
      • decreased arterial oxygen tesion when upright and improves when supine
    • signs of liver disease
      • spider angiomata
      • palmar erythema
      • gynecomastia
      • jaundice
    • spider nevi (spider angiomas) 
  • Transthoracic contrast echocardiography
    • indications
      • in patients who have evidence of impaired arterial oxygention (e.g., increased A-a gradient)
      • considered gold standard for detecting intrapulmonary vascular dilatation and diagnosing hepatopulmonary syndrome (HPS)
    • finding
      • intrapulmonary vascular dilatation
  • Labs
    • arterial blood gas (ABG) analysis
      • indications
        • when there is clinical suspicion for hepatopulmonary syndrome
          • ≥ 15 mmHg is suggestive of HPS
          • ≥ 20 mmHg is suggestive of HPS in patients > 64-years-old
  • Arteriovenous malformations
  • Atrial septal defect
  • Hepatic hydrothorax
  • Portopulmonary hypertension
  • Anemia
  • Conservative
    • observation
      • indication
        • in patients with mild-to-moderate HPS
    • long-term oxygen therapy
      • indication
        • in patients with severe HPS
  • Operative
    • liver transplantation
      • indications
        • in patients with severe HPS
  • Pneumonitis
  • Panacinar emphysema

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