Lymphangioleiomyomatosis (LAM) is a rare diffuse cystic lung disease.Knowledge on LAM-related pulmonary hypertension (PH) is limited.This study aimed to analyze the clinical characteristics of LAM with elevated pulmonary artery pressure (PAP) and evaluate the potential efficacy of sirolimus.The study involved 50 LAM patients who underwent echocardiography.According to the tricuspid regurgitation velocity (TRV),these patients were divided into the TRV ≤ 2.8 m/s group and TRV ＞ 2.8 m/s group.Both groups comprised 25 females with an average age of 38.6 ± 8.1 and 41.5 ± 8.9 years.In the TRV ＞ 2.8 m/s group,the estimated systolic PAP (SPAP) was significantly elevated (52.08 ± 12.45 mmHg vs.30.24 ± 5.25 mmHg,P ＜ 0.01).Linear analysis showed that SPAP was correlated with forced expiratory volume in 1 s (FEV1),diffusing capacity of the lungs for carbon monoxide,alveolar arterial oxygen gradient (PA-aO2),and 6 min walking distance (r =-0.392,-0.351,0.450,and-0.591,respectively;P ＜ 0.05),in which PA-aO2 was a risk factor for SPAP elevation (β =0.064,OR =1.066,P ＜ 0.05).Moreover,in 10 patients who received sirolimus therapy,SPAP decreased from 57.0 ± 12.6 mmHg to 35.2 ± 11.1 mmHg.The study showed that LAM patients with PH exhibit poor pulmonary function and hypoxemia and may benefit from sirolimus treatment.