Hepatopulmonary syndrome (HPS) is characterized by the triad of liver disease, intrapulmonary vascular dilatation and arterial deoxygenation. Mediating factors are tumor necrosis factor a, endothelin 1 and nitric oxide. Typical symptom is an increase in dyspnea while in standing position, orthodeoxia. In orthodeoxia hypoxemia accentuates while the partial pressure of oxygen in arterial blood decreases by 5% or more. The blood flow distribution to the lungs is changed as the pulmonary vascular tone is altered. Diagnosis is based on the evidence of liver disease, hypoxemia and pulmonary vascular shunt detected by the so-called bubble test. Liver transplantation is currently the only efficient therapeutic option.
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