COMBINED PORTAL AND PULMONARY-HYPERTENSION IN SARCOIDOSIS

A case is reported of a 48-year-old man previously diagnosed as having mitral valvular disease, who was admitted for evaluation of chronic c or pulmonale. Seven years before admission, an intraoperative liver bi opsy had shown multiple noncaseating granulomas. The further course wa s characterized by progressive chronic intrahepatic cholestasis and po rtal hypertension. Right heart catheterization revealed a mean pulmona ry artery pressure of 43 mm Hg and a normal wedge pressure (5 mm Hg). A perfusion lung scan was normal. Open lung biopsy demonstrated noncas eating granulomas and extensive pulmonary fibrosis. To our knowledge, only one case of sarcoidosis with combined portal and pulmonary hypert ension has previously been described.