摘要目的 分析肺静脉闭塞病(PVOD)的临床表现、诊断治疗及预后情况.方法 回顾性分析2008年5月至2011年5月间同济大学附属上海市肺科医院临床诊断PVOD患者的临床特点及预后情况.结果 共5例患者被诊断为PVOD,年龄范围为12 ～42(22±12)岁,其中女性4例,男性1例.患者从首发症状至被确诊为PVOD的时间为2～50(16±20)个月,其中4例曾被误诊为特发性肺动脉高压.患者确诊时心功能均显著下降,其中3例为WHO肺高压功能分级Ⅲ级,另2例为Ⅳ级.所有患者胸部CT检查均提示双肺弥漫性毛玻璃样渗出征象,肺功能检查示肺泡弥散功能显著下降,平均肺泡一氧化碳弥散量/预计值为(38±12)％.右心导管测得患者肺动脉压力和肺血管阻力均显著升高.5例患者均接受传统和肺动脉高压靶向药物治疗.随访至今,其中4例患者因难治性右心衰竭死亡,从出现首发症状至死亡及从确诊PVOD至死亡的时间分别为5～65(27±26)个月和1 ～16(9±9)个月.结论 PVOD是罕见的恶性心肺血管疾病,易被误诊,使用现有肺动脉高压靶向治疗药物疗效不佳、预后差,应及早考虑进行肺移植治疗.

abstractsObjective To investigate the clinical presentation,diagnosis,treatment and outcome of patients with pulmonary veno-occlusive disease (PVOD).Methods Data from patients diagnosed as PVOD from May 2008 to May 2011 in Shanghai Pulmonary Hospital,Tongji University were retrospectively reviewed.Results During this period,5 patients [ 4 female,aged from 12 to 42 (22 + 12 ) years old ] were diagnosed as PVOD.The durations from symptoms onset to PVOD diagnosis was 2 to 50 ( 16 ± 20)months and four of them were previously diagnosed as idiopathic pulmonary arterial hypertension.All patients at the time of PVOD diagnosis had a severely impaired WHO pulmonary hypertension functional class (3 in class Ⅲ and 2 in class Ⅳ ).Furthermore,all patients characterized by a typical sign of centrilobular ground-glass opacities in high-resolution computed tomography,a markedly reduction of diffusing capacity of the lung for carbon monoxide [ (38 ± 12)％ of predicted value] in pulmonary functional test and severely compromised cardio-pulmonary hemodynamics identified by right heart catheterization.All patients received conventional and pulmonary arterial hypertension specific therapies,and then followed-up regularly.Up to now,4 out of 5 patients died due to refractory right heart failure.The durations from symptoms onset to death and from PVOD establish to death were 5 - 65 ( 27 + 26 ) months and 1 - 16 (9 ± 9 ) months,respectively.Conclusions PVOD is a rare and malignant cardio-pulmonary disorder that often be misdiagnosed as idiopathic pulmonary arterial hypertension.Given the poor responses to modem pulmonary arterial hypertension specific therapies,lung transplantation remains the treatment of choice.