Xiong Xianliang, Zhu Tengteng, Li Jiang
Department of Cardiology, Second Xiangya Hospital, Central South University, Changsha 410011, China.
Zhong Nan Da Xue Xue Bao Yi Xue Ban. 2018 May 28;43(5):571-576. doi: 10.11817/j.issn.1672-7347.2018.05.017.
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 findings, lung function, bronchoscopy and other resources. There 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.
肺静脉闭塞性疾病(PVOD)/肺毛细血管瘤病(PCH)是一种导致肺动脉高压的罕见肺血管疾病形式。PVOD/PCH的诊断可通过临床特征、体格检查、影像学表现、肺功能、支气管镜检查及其他资料综合判断。目前尚无针对PVOD/PCH的确立的药物治疗方法,PVOD/PCH唯一的治愈性治疗方法是肺移植。一名患有PVOD/PCH的女孩在中南大学湘雅二医院被确诊。结合该病例特点及相关文献,我们总结了该疾病的流行病学、病因、诊断及治疗方法,以提高医生对这种罕见疾病的认识。