Budd-Chiari症候群による肝硬変に合併した肺高血圧症の1剖検例

An Elderly Case fo Budd-Chiari Syndrome Associated with Pulmonary Hypertension

　症例は65歳，男性．20歳時に腹壁静脈の怒張に気づき，44歳時に血管造影検査などによりBudd-Chiari症候群による肝硬変と診断された．63歳時には肝細胞癌の合併も確認されたが，肝予備能の低下のため侵襲的治療は施行されなかった．65歳時に肝腎不全のため死亡した．剖検にて疎性結合織による肝部下大静脈の膜様閉鎖が確認された．肺の組織像ではHeath-Edwards分類のgradeVに相当する肺高血圧性血管病変が認められた．肝臓は典型的な肝硬変の形態を示し，肝細胞癌がその4／5を占拠していた．われわれが調べ得た限りではこれまでにBudd-Chiari症候群と肺高血圧症の合併報告はない．本症例は長期間にわたってBudd-Chiari症候群の自然歴ともいえる経過を観察できた点でも興味深い．

We described a male patient with pulmonary hyper-tension due to Budd-Chiari syndrome who died at the age of 65 years. At the age of 20, he had complained of the dilatation of veins on the surface of the abdominal wall. At the age of he had experienced hematemesis and loss of consciousness and on the basis of angiogra-phic findings had been diagnosed as having Budd Chiari syndrome. At the age of 62, right sided cardiac catheter-ization revealed precapillary pulmonary hypertension (with a mean pulmonary arterial pressure of 52mmHg) but there was no evidence of intracardiac shunts. At the age of 63, he had hepatocellular carcinoma, and he died of hepato renal failure at the age of 65. On autopsy, complete membranous occlusion of the hepatic portion of the inferior versa cava by the loose connective tissuewas confirmed. Histopathologic examination disclosed hypertensive pulmonary vascular disease equivalent to grade V of the classification by Heath and Edwards. In addition. hepatocellular carcinoma occupied four fifths of the liver. The exact mechanism of pulmonary hyper-tension associated with Budd Chiari syndrome remained to be determined. Many papers have reported an association between pulmonary hypertension and liver cirrhosis. To our knowledge, this is the first case of Budd-Chiari syndrome complicated with pulmonary hypertension. We expect this case to be helpful for consideration of the relationship of these two distinct diseases.