Pulmonary hypertension following hepatopulmonary syndrome in a patient with cirrhosis

We report the case of a patient with liver cirrhosis who successively devel oped hepatopulmonary syndrome and portopulmonary hypertension. Initially, t he patient presented with severe dyspnea and hypoxemia at rest, Technetiun- 99 macroaggregated albumin lung perfusion scan demonstrated right-to-left s hunt, and hemodynamic study revealed a hyperdynamic, state with low pulmona ry vascular resistance, thus confirming the diagnosis of hepatopulmonary sy ndrome. More than 2 years after the onset of pulmonary symptoms, a marked i mprovement in dyspnea and gas exchange was observed, Lung perfusion scan di d not disclose any right-to-left shunt and right-sided heart catheterizatio n showed evidence of severe pulmonary hypertension, We conclude that hepato pulmonary syndrome and portopulmonary hypertension are not mutually exclusi ve, We hypothesize that, by reversing the phenomenon of intrapulmonary vaso dilatation, the development of portopulmonary hypertension interfered with each of the potential causes of hypoxemia in hepatopulmonary syndrome (vent ilation-perfusion inequalities, intrapulmonary shunting, oxygen diffusion l imitation) and, as a result, led to a correction of hypoxemia.