Pulmonary Vascular Complications of Liver Disease

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Patients living with liver disease are at risk for conditions involving the pulmonary vasculature. Venous blood passing through the liver flows “downstream” to the lungs, and dysfunction of either organ can profoundly affect the other. The cardinal pulmonary vascular syndromes resulting from liver dysfunction are hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH). Pathophysiologically, they could be considered as opposite ends of the spectrum, with HPS causing dilation and POPH causing constriction/obstruction of the pulmonary vascular bed. Development of these syndromes is associated with increased morbidity and mortality,1,2 and can correlate surprisingly poorly with the measurable severity of liver impairment.3–5 As recognition and understanding of these conditions grow, so do treatment approaches. In particular, the role of liver transplantation (LT) in patients with pulmonary vascular complications has been debated.6,7 Experience has shown that both groups of patients can have resolution of their pulmonary vascular disease following LT.8,9 This article will examine current understanding of and trends in management of the pulmonary vascular complications of liver disease.

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