Hepatopulmonary syndrome

Refers to a defect in arterial oxygenation induced by pulmonary vascular dilation associated with liver disease.

Three aspects of the disease include liver disease, pulmonary vascular dilation and an oxygenation defect.

Classification based on severity of oxygenation defect as it influences survival and timing of liver transplantation.

Vascular dilation includes pulmonary capillaries and less commonly pleural and pulmonary arteriovenous communications.

There is an increase in the alveolar-arterial gradient on room air and vascular dilations causing right to left shunting.

Associated with spider angiomata, platypnea (difficulty in breathing when erect, relieved by lying down), and orthodeoxia (increasing arterial oxygen desaturation with the erect position).

Diagnosis confirmed by contrast enhanced echocardiography or microaggregated technetium labeled albumin scanning.

Treatment is liver transplantation.

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