肺静脉堵塞病：1例报道及文献复习  

Pulmonary veno-ocelusive disease： one case report and literature review

作　　者：牟向东[1] 李海潮[1] 李桂莲[1] 王广发[1] 阙呈立[1] 何冰[1] 

机构地区：[1]北京大学第一医院呼吸内科,100034

出　　处：《国际呼吸杂志》2008年第3期133-137,共5页International Journal of Respiration

摘　　要：目的提高对肺静脉堵塞病（PVOD）的认识。方法对1例PVOD患者的临床资料进行分析，并结合文献复习。结果PVOD是一种临床少见病，临床表现为进行加重的劳力性呼吸困难和肺动脉高压。影像学特征为肺水肿、Kerley B线、肺动脉高压和右心房室扩大等征象，左心房正常。血流动力学表现为肺动脉压升高，肺动脉楔压（PAWP）和左房压正常。组织病理学可见弥漫性的肺小静脉管腔狭窄和闭塞。结论重度肺动脉高压、肺水肿和PAWP正常（或左心房内径正常）被称为PVOD三联征，可作为PVOD的临床诊断标准。血管扩张剂可诱发急性肺水肿和猝死，应谨慎使用。PVOD预后不良，肺移植是惟一确定有效的治疗方法。Objective To highlight the characteristics of pulmonary veno-occlusive disease（PVOD）. Methods One patient with PVOD was presented and the literature on the subject was reviewed. Results PVOD is a rare cause of pulmonary hypertension characterized with progressive dyspnea on exertion. Radiological examination shows pulmonary edema, Kerley B lines, pulmonary hypertension and enlargement of right ventricle, left atrium is normal. Hemodynamic features include pulmonary hypertension, normal pulmonary artery wedge pressure （PAWP） and normal left atrial pressure. The main pathological findings are extensive and diffuse occlusion of pulmonary veins. Conclusions The clinical diagnostic criterion is the presence of the classic triad of severe pulmonary arterial hypertension, radiographic evidence of pulmonary edema,and normal PAWP or normal left atrium. Vasodilator treatment carries a risk of acute pulmonary edema and should be undertaken with caution. The prognosis is poor and lung transplantation remains the sole curative therapy for PVOD.

关 键 词：肺动脉高压 肺静脉堵塞病 肺移植 

分 类 号：R563.5[医药卫生—呼吸系统]