Hepatopulmonary syndrome associated with cardiorespiratory disease

Background/Aims: Hepatopulmonary syndrome is defined as a clinical triad in cluding chronic liver disease, abnormal pulmonary gas exchange resulting ul timately in profound arterial hypoxaemia, and evidence of intrapulmonary va scular dilatations, We report five patients with liver cirrhosis diagnosed with hepatopulmonary syndrome who had associated chronic obstructive or res trictive respiratory diseases. Methods: Clinical, radiographic and constrast-enhanced echocardiographic fi ndings, and systemic and pulmonary haemodynamic and gas exchange, including ventilation-perfusion distributions, measurements were assessed in all fiv e patients. Results: Echocardiography was consistent with the presence of intrapulmonar y vasodilation without intracardiac abnormalities, and high resolution comp uted tomographic scan features were compatible with clinical (3 cases) or h istopathological diagnoses (2 cases) of associated respiratory disorders. T he most common prominent functional findings were moderate to severe arteri al hypoxaemia, caused by moderately to severely increased intrapulmonary sh unting and/or mild to moderate low ventilation-perfusion areas, and hypocar bia along with an increased cardiac output and a low pulmonary artery press ure and vascular resistance, Conclusions: These functional characteristics, classically reported in the setting of clinically stable, uncomplicated hepatopulmonary syndrome, confo rm to a distinctively unique, chronic gas exchange pattern. Equally importa nt, these pulmonary haemodynamic-gas exchange hallmarks are not influenced by the coexistence of chronic cardiorespiratory disease states. These data may have clinical relevance for elective indication of hepatic transplantat ion in patients with life-threatening hepatopulmonary syndrome.