Hepatopulmonary syndrome

Anamnesis: We report about a 60-year-old woman with hepatopulmonary syndrom e. The Patient suffered from progressive dyspnea and liver cirrhosis follow ing viral hepatitis C, acquired through blood transfusion 30 years ago. Examination Results: Remarkable were clinical signs of chronic liver diseas e (cutaneous spider naevi, palmar erythema) and signs of chronic respirator y failure (clubbing) with platypnea Arterial PO, was 33 mm Hg under 41O(2)/ min nasal oxygen supplement and improved to 74 mm Hg under 81O(2)/min. Arte rial pO(2) deteriorated in upright position (orthodeoxia). Contrast enhance d two-dimensional transthoracic echocardiography showed a delayed microbubb le opacification in the left heart chambers (without atrial or ventricular septal defect), suggesting pulmonary vascular dilatations. Perfusion scan w ith macroaggregated : albumin labelled with technetium-99m showed uptake ov er thyroid, kidneys, liver, spleen suggesting an abnormal passage through t he pulmonary vascular bed. The radiological examination with chest radiogra phy and high resolution computer tomography revealed no signs of interstiti al lung disease. The pulmonary. artery angiography showed neither anatomica l shunts nor malformations. Diagnosis: The final diagnosis was hepatopulmonany syndrome Type 1. Treatment/Follow-Up: The patient underwent liver transplantation with follo wing good function of the liver graft. After 6 months the patient presented in a much better general condition, but with persisting severe hypoxemia w hereas the spider naevi disappeared completely. One year after the liver tr ansplantation in the perfusion scan the uptake river thyroid, kidneys, live r, and spleen was absent. The orthodeoxia and hypoxemia improved evidently. From that we conclude that the pulmonary vascular dilatations diminished a fter liver transplantation.