Hepatopulmonary Syndrome and Portopulmonary Hypertension in Children: Recent Advances in Diagnosis and Management

Pulmonary vascular disease is an important complication of portosystemic shunting in children and adults with chronic liver diseases and cirrhosis, extrahepatic portal vein obstruction/thrombosis or congenital portosystemic shunts (CPSSs).1 Two major types of pulmonary vascular disease in this context are hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH),1 both of which may indicate the need for liver transplantation.2 The hallmark of HPS is intrapulmonary vascular dilatation (IPVD) and shunting leading to an increased alveolar-arterial gradient and, generally, hypoxemia (Table I).

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