Veeraraghavan S, Koss M N, Sharma O P
Division of Pulmonary and Critical Care Medicine, University of Southern California School of Medicine, Los Angeles 90033, USA.
Curr Opin Pulm Med. 1999 Sep;5(5):310-3. doi: 10.1097/00063198-199909000-00008.
Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension that mainly affects children and young adults. Its cause is unknown, although viral infections and drugs have been implicated. Patients with PVOD present with symptoms of right-sided heart failure. Radiologic examination shows prominent pulmonary arteries with Kerley B lines, pleural effusion, and mediastinal adenopathy. The definite diagnosis is made by histologic examination. Eccentric intimal fibrosis and recanalized thrombi in pulmonary veins and venules, arterialized veins, alveolar edema, and medial hypertrophy of arteries are seen on lung biopsy. No effective treatment is available; lung transplantation has been tried. The prognosis associated with PVOD is poor.
肺静脉闭塞性疾病（PVOD）是一种导致肺动脉高压的罕见病因，主要影响儿童和年轻人。其病因不明，尽管病毒感染和药物被认为与之有关。PVOD患者表现为右心衰竭症状。放射学检查显示肺动脉突出，伴有克氏B线、胸腔积液和纵隔淋巴结肿大。通过组织学检查做出明确诊断。肺活检可见肺静脉和小静脉的偏心性内膜纤维化和再通血栓、动脉化静脉、肺泡水肿以及动脉中层肥厚。目前尚无有效治疗方法；已尝试进行肺移植。与PVOD相关的预后很差。
