Pulmonary Hypertension in Pulmonary Lymphangiomyomatosis:a Case Report
10.3969/j.issn.1000-3614.2024.08.014
- VernacularTitle:肺淋巴管肌瘤病相关肺动脉高压一例
- Author:
Lixing HU
1
;
Qin LUO
;
Zhihui ZHAO
;
Li DENG
;
Qing ZHAO
;
Zhihong LIU
Author Information
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
- Keywords:
pulmonary lymphangiomyomatosis;
pulmonary cystic lesion;
hypoxemia;
pulmonary hypertension
- From:
Chinese Circulation Journal
2024;39(8):819-821
- CountryChina
- Language:Chinese
-
Abstract:
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.
