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A 38 year old man, submitted for orthotopic liver transplantation for α1 antitrypsin deficit hepatic cirrhosis, was admitted to the intensive care unit for postoperative monitoring. Ventilatory weaning was difficult because of severe hypoxaemia even using high FIo2 (Pao2 < 60 mm Hg for FIo2 of 0.7). After extubation the patient remained hypoxaemic with Pao2 of 60 mm Hg and breathing 50% supplementary oxygen. Orthodeoxia was not evident but contrast enhanced transthoracic echocardiography showed delayed microbubble opacification of left heart chambers suggestive of hepatopulmonary syndrome (panels A–C).
Hepatopulmonary syndrome is a pulmonary complication of advanced chronic liver disease, characterised by the presence of severe hypoxaemia (Pao2 < 60 mm Hg breathing room air), dyspnoea, platypnoea, orthodeoxia, and digital clubbing. The main physiopathologic explanation for the syndrome is the presence of arteriovenous pulmonary shunting related to intrapulmonary vascular abnormalities akin to cutaneous spider angiomata found in cirrhotic patients. These abnormalities are mostly present in the lower pulmonary fields justifying orthodeoxia (a fall in arterial oxygen tension upon assuming an upright position). Hypoxaemia may also be explained by other mechanisms such as perfusion-diffusion defects, failure of hypoxic pulmonary vasoconstriction, change in mediators of vascular dilatation, or rightwards displacement of the O2–haemoglobin dissociation curve.
Up to 80% of patients with hepatopulmonary syndrome show improvement or resolution 15 months after orthotopic liver transplantation.
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