Chronological change in pulmonary vascular response to hypoxia in hepatopulmonary syndrome.

Here we present a case of hepatopulmonary syndrome (HPS) where spontaneous resolution of severe hypoxaemia occurred with the development of pulmonary hypertension over several years after the initial diagnosis of HPS. The pulmonary vascular responses to hypoxia examined before and after the spontaneous resolution of HPS confirmed that the pathogenesis of HPS could be functional and reversible in nature. To the best of our knowledge, this is the first report demonstrating a remarkable change in the pulmonary vascular response to hypoxia before and after the spontaneous resolution of hypoxaemia in HPS.