Ito M, Honda M, Shikuwa S, Kawase Y, Matsumoto K, Ohi J, Sekine I, Fujii H
Department of Pathology, Nagasaki University School of Medicine, Japan.
Acta Pathol Jpn. 1987 Dec;37(12):1935-43. doi: 10.1111/j.1440-1827.1987.tb03307.x.
Two autopsy cases of pulmonary hypertension (PH) associated with liver cirrhosis are presented. Both patients were hepatitis B (HB) virus carriers and suffered from type B cirrhosis during the clinical course. The first patient was a 52-year-old male with type B cirrhosis. He died of hepatic encephalopathy but did not have any specific symptoms for PH except abnormal laboratory findings. Chest roentgenograms displayed prominence of the central pulmonary artery. Cardiac catheterization indicated marked increment of pulmonary arterial pressure. Autopsy revealed dilatation and sclerosis of the main pulmonary artery and right ventricular hypertrophy. Microscopically, the pulmonary arteries showed intimal fibrosis, medial hypertrophy, and plexiform lesions throughout the lungs. The second patient, a 15-year-old boy, had PH with juvenile liver cirrhosis which had existed for 8 years prior to the onset of PH. He complained of severe dyspnea and dizziness before death. Electrocardiogram indicated right ventricular hypertrophy. Autopsy disclosed cardiomegaly, type B cirrhosis and sclerotic pulmonary arteries. Grade VI pulmonary plexogenic arteriopathy including plexiform lesions and necrotizing arteritis was observed. HBsAg was detected in both the hepatocytes and the pulmonary arterial walls. We discuss the possible relationship between persistent HB viral infection and PH with liver cirrhosis.
本文报告两例与肝硬化相关的肺动脉高压（PH）尸检病例。两名患者均为乙型肝炎（HB）病毒携带者，临床过程中均患有乙型肝硬化。首例患者为一名52岁男性，患有乙型肝硬化。他死于肝性脑病，但除实验室检查异常外，无任何PH的特异性症状。胸部X线片显示中央肺动脉突出。心导管检查显示肺动脉压显著升高。尸检发现主肺动脉扩张和硬化，右心室肥厚。显微镜下，肺动脉显示内膜纤维化、中膜肥厚，全肺可见丛状病变。第二例患者为一名15岁男孩，患有PH合并青少年肝硬化，在PH发病前已存在8年。他在死前抱怨严重呼吸困难和头晕。心电图显示右心室肥厚。尸检发现心脏肿大、乙型肝硬化和硬化的肺动脉。观察到VI级肺丛状动脉病，包括丛状病变和坏死性动脉炎。在肝细胞和肺动脉壁中均检测到HBsAg。我们讨论了持续性HB病毒感染与肝硬化相关PH之间的可能关系。
