肺淋巴管肌瘤病相关肺动脉高压一例  

Pulmonary Hypertension in Pulmonary Lymphangiomyomatosis:a Case Report

作　　者：胡立星 罗勤 赵智慧 邓丽 赵青 柳志红 HU Lixing;LUO Qin;ZHAO Zhihui;DENG Li;ZHAO Qing;LIU Zhihong(Respiratory and Pulmonary Vascular Diseases Center,State Key Laboratory of Cardiovascular Disease,National Center for Cardiovascular Diseases and Fuwai Hospital,Chinese Academy of Medical Sciences and Peking Union Medical College,Beijing 100037,China)

机构地区：[1]中国医学科学院、北京协和医学院、国家心血管病中心、阜外医院、心血管疾病国家重点实验室、呼吸与肺血管病中心,北京100037

出　　处：《中国循环杂志》2024年第8期819-821,共3页Chinese Circulation Journal

基　　金：北京医学奖励基金(632 T2022-ZX020)。

摘　　要：肺淋巴管肌瘤病(LAM)是一种罕见的主要发生于育龄期女性的慢性进展性弥漫性囊性肺疾病,肺动脉高压是LAM的一种少见并发症,目前LAM相关肺动脉高压在流行病学、发病机制、治疗策略等方面证据匮乏。本文报道一例表现为气短的育龄期女性,结合特异性肺部影像学表现及血清血管内皮生长因子-D诊断为LAM,经右心导管检查证实为毛细血管前性肺动脉高压,经西罗莫司治疗及家庭氧疗病情稳定。Pulmonary lymphangiomyomatosis(LAM)is a rare chronic progressive diffuse cystic lung disease that mainly occurs in women of reproductive age.Pulmonary hypertension is a rare complication of LAM.Currently,there is insufficient evidence on the epidemiology,pathogenesis and treatment strategy of LAM related pulmonary hypertension.We reported a case of a woman at reproductive age with shortness of breath and diagnosed with LAM by the combination of specific lung imaging features and serum vascular endothelial growth factor D.Precapillary pulmonary hypertension was confirmed by right cardiac catheterization.Her condition was stable with Sirolimus and home oxygen therapy.

关 键 词：肺淋巴管肌瘤病 肺囊性病变 低血氧症 肺动脉高压 

分 类 号：R54[医药卫生—心血管疾病]