肺静脉闭塞病/肺毛细血管瘤样病1例及文献复习

肺静脉闭塞病(pulmonary veno-occlusive disease，PVOD)/肺毛细血管瘤样病(pulmonary capillary hemangiomatosis，PCH)是临床上罕见的引起肺动脉高压的一类肺血管疾病，其诊断需要综合临床表现、影像、肺功 能、支气管镜等多方面的资料，治疗上尚无明确有效的药物，肺移植是目前唯一有效的治疗方案。中南大学湘雅二 医院收治1例PVOD/PCH患者，现结合病例特点及相关文献报道，对该病的流行病学、病因、诊断和治疗进行介绍， 以提高临床医生对这一罕见病的认识。

Pulmonary veno-occlusive disease/pulmonary capillary hemangiomatosis: A case report and literature review

Pulmonary veno-occlusive disease (PVOD)/pulmonary capillary hemangiomatosis (PCH) is a rare form of pulmonary vascular disease that causes pulmonary arterial hypertension. The diagnosis of PVOD/PCH can be established by the combination of clinical features, physical examination, radiological fi ndings, lung function, bronchoscopy and other resources. Th ere is no established medical therapy for PVOD/PCH, and the only curative therapy for PVOD/PCH is lung transplantation. A girl with PVOD/PCH was diagnosed in the Second Xiangya Hospital. Combining the characteristics for this case with the relevant literature, we summarized the epidemiology, etiology, diagnosis and treatment for the disease to raise doctors’ awareness for this rare disease.