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Hepatopulmonary Syndrome and Portopulmonary Hypertension

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Hepatopulmonary Syndrome and Portopulmonary Hypertension

Transition and Coexistence of HPS and PPHT


In contrast to what might be expected based on their opposite underlying pathogenesis and pathophysiology, HPS and PPHT are not mutually exclusive. Case reports described the possible transition between HPS and PPHT or coexistence of HPS and PPHT in the same patient. One hypothesize that this may be caused by (i) dysregulation of a shared vascular signalling pathway, which may lead to either pulmonary vasoconstriction or vasodilation, or (ii) preferential binding of ET-1 to either the ET-1A or the ET-1B receptor, resulting in vasoconstriction or vasodilation respectively.

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