Clinical characteiristics and survival of patients with pulmonary veno-occlusive disease
10.3760/cma.j.issn.0253-3758.2011.10.005
- VernacularTitle:肺静脉闭塞病患者的临床特点及预后分析
- Author:
Xin JIANG
1
;
Fa-Dong CHEN
;
Jing HE
;
Rong JIANG
;
Ruo-Min DI
;
Qin-Hua ZHAO
;
Zhi-Cheng JING
Author Information
1. 同济大学附属上海市肺科医院
- Keywords:
Hypertension,pulmonary;
Pulmonary veno-occlusive disease
- From:
Chinese Journal of Cardiology
2011;39(10):896-900
- CountryChina
- Language:Chinese
-
Abstract:
Objective 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.
