[Clinical characteristics and survival of patients with pulmonary veno-occlusive disease].
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 modern pulmonary arterial hypertension specific therapies, lung transplantation remains the treatment of choice.
Department of Cardio-Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University, Shanghai 200433, China.
Xin
X
Jiang
Fa-dong
FD
Chen
Jing
J
He
Rong
R
Jiang
Ruo-min
RM
Di
Qin-hua
QH
Zhao
Zhi-cheng
ZC
Jing
D000293:Adolescent; D000328:Adult; D006328:Cardiac Catheterization; D002681:China; D003951:Diagnostic Errors; D065627:Familial Primary Pulmonary Hypertension / Q000473:pathology / Q000628:therapy*; D005260:Female; D006439:Hemodynamics; D006801:Humans; D008168:Lung; D016040:Lung Transplantation; D008297:Male; D011668:Pulmonary Veno-Occlusive Disease / Q000175:diagnosis / Q000473:pathology / Q000628:therapy*; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed; D055815:Young Adult