Hepatopulmonary Syndrome (HPS) is a condition characterized by pulmonary vascular dilation, abnormal {|###|}stirred pulse{|###|} oxygenation, and hypoxemia occurring in liver disease. This syndrome was first reported by Rydell Hoffbauer in 1956, and the concept of HPS was proposed by Kennedy and Knudson in 1977. Hepatopulmonary Syndrome is primarily observed in patients with severe cirrhosis (Child-Pugh class C), often accompanied by massive {|###|}ascites{|###|}, clubbing fingers, portal hypertension, and insufficient oxygen supply due to {|###|}stirred pulse{|###|}. PaO₂ is often <10 kPa.

bubble_chart Pathogenesis

The pathogenesis of hepatopulmonary syndrome is related to decreased affinity of oxyhemoglobin, pulmonary capillary dilation caused by vasodilators such as prostaglandins, intrapulmonary (arteriovenous and portopulmonary venous) shunting, impaired oxygen diffusion between alveoli and capillaries, ventilation/perfusion mismatch, and compression from conditions like ascites. The main pathological changes include pulmonary vascular dilation, pulmonary circulation disorders, multiple anastomotic branches between peripheral pulmonary vascular beds and large hilar arteries and veins, allowing mixed venous blood to enter pulmonary veins.

bubble_chart Clinical Manifestations

The main features are difficulty breathing and cyanosis.

bubble_chart Diagnosis

In the diagnosis of chronic liver diseases, especially in patients with cirrhosis and massive ascites, severe hypoxemia (PaO₂ < 6.7 kPa) should raise suspicion of this syndrome. A PaO₂ < 10 kPa is a necessary condition for diagnosing HPS; orthodeoxia is a sensitive and specific indicator for diagnosing HPS.

bubble_chart Treatment Measures

In treatment, the first priority should be addressing hypoxemia by providing oxygen, which can be administered via nasal cannula at 2-3L/min. The efficacy of glucocorticoids, somatostatin, and prostaglandin inhibitors, as well as their applications, still require further research for confirmation.