Hepatopulmonary syndrome history and symptoms

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Soroush Seifirad, M.D.[2]

Overview

The hallmark of hepatopulmonary syndrome is platypnea, and orthodeoxia . A positive history of liver disease and dyspnea is suggestive of hepatopulmonary syndrome. Other sign and symptoms of hepatopulmonary syndrome may include spider angiomata, clubbing of fingers or toes, and cyanosis

History and Symptoms

• The majority of patients with hepatopulmonary syndrome (80 percent) initially present with their liver disease symptoms. The remained 20 percent present with pulmonary symptoms. The most complication and prsentation is an insidiously progressive shortness of breath (dyspnea) at rest or upon exertion.

• The hallmark of hepatopulmonary syndrome is platypnea (dyspnea in upright position which is improved in supine position), and orthodeoxia (a drop of 4mmHg in PaO2 or 5% in saturation when moving from the supine to the standing position). Almost 90 percent of patients with HPS present with this specific finding.
• Other sign and symptoms of hepatopulmonary syndrome may include spider angiomata, clubbing of fingers or toes, and cyanosis.

History

Patients with hepatopulmonary syndrome may have a positive history of:

• liver disease

• Either severe or mild disease could be complicated with HPS.
• Although most of the patients with HPS suffer from chronic liver disease, it might happen in a patient with acute liver disease as well.
  • History of infections:
    • Hepatitis B
    • Hepatitis C
  • History of autoimmune disorders:
    • autoimmune hepatitis
    • Crohn's disease
  • History of blood transfusions
    

• Dyspnea
• Unexplained decreased O2 Saturation below 96 percent.
• Platypnea
• Orthodeoxia

Common Symptoms

Common symptoms of hepatopulmonary syndrome include:

• Dyspnea
• Platypnea
• Clubbing
• Cyanosis
• Symptoms of liver disease like:

• Spider nevi

• • Symptoms of hypogonadism:
    • • Impotence
      • Infertility
      • Testicular atrophy
      • Loss of libido
    • Easy bruisability
  • Decompensated cirrhosis presentations such as:
    • Jaundice
    • Increase in abdominal girth due to ascites
    • Pruritus
    • Signs of upper gastrointestinal bleeding:
      • Hematemesis
      • Hematochezia
      • Melena
    • Symptoms due to hepatic encephalopathy:
      • Altered mental status
      • Confusion
      • Sleep disturbances
    • Muscle cramps due to reduction in effective circulating plasma volume
    • Lower extremity edema

Less Common Symptoms

Less common symptoms of hepatopulmonary syndrome include

• Clubbing
• Cyanosis