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目的:探讨淋巴管肌瘤病相关肺动脉高压（LAM-PH）患者的临床特征，并评估肺动脉光学相干断层扫描术（OCT）在协助PH病因诊断方面的价值。方法:回顾性分析2017年6月至2023年8月在广州医科大学附属第一医院广州呼吸疾病研究所单中心就诊的234例LAM患者资料，其中167例行超声心动图检查，纳入15例超声心动图提示存在肺动脉高压的患者作为LAM-PH组，从剩余的152例患者中随机选取32例患者作为对照组，对比两组患者的人口学信息、临床表现、肺功能、血气分析和血清VEGF-D水平，并进一步评估5例患者右心导管数据以及2例患者肺动脉OCT检查结果。结果:167例LAM中15例（8.98%）存在肺动脉高压。LAM-PH组接受氧疗的比例高于对照组（86.7%比46.9%， P=0.01）。LAM-PH组FEV 1占预计值%、FVC占预计值%、FEV 1/FVC占预计值%、D LCO占预计值%均明显低于对照组（ P值分别为0.001、<0.001、0.011及0.001）。LAM-PH组中有5例患者进行了右心导管检查，5例患者均为毛细血管前性肺动脉高压，3例患者肺动脉压力重度升高。LAM-PH组血清VEGF-D水平（2 415.30±1 140.47 ng/L）显著高于对照组［（1 353.67±923.89）ng/L， P=0.004］。2例患者肺动脉OCT检查提示血管内膜未见明显异常，管腔内无血栓和增生物。 结论:LAM患者中PH的患病率较低，LAM-PH组的患者表现出更加严重的呼吸困难、低氧血症、肺通气功能及弥散功能障碍。LAM-PH患者血清VEGF-D水平显著高于对照组患者。LAM患者伴发的肺动脉高压以毛细血管前性为主。肺动脉OCT检查可评估肺动脉血管情况，有助于排查LAM患者发生肺动脉高压病因。

Objective:To describe the prevalence of lymphangioleiomyomatosis-pulmonary hypertension (LAM-PH), to explore the clinical features of patients with LAM-PH, and to evaluate the role of pulmonary artery optical coherence tomography (OCT) in the diagnosis of LAM-PH.Methods:Among 234 patients diagnosed with LAM in our center from June 2017 to August 2023, echocardiography was performed in 167 patients, 15 patients with PH indicated by echocardiography were selected as the LAM-PH group. From the remaining 152 patients, 32 patients were randomly selected as the control group. We compared the demographic data, clinical manifestations, pulmonary function, blood gas analysis, and serum vascular endothelial growth factor D (VEGF-D) levels between the two groups. We also evaluated the data from right heart catheterization in five patients and the images from optical coherence tomography of pulmonary arteries in two patients.Results:Echocardiography showed pulmonary hypertension in 15 patients (8.98%). Compared with the control group, LAM-PH group had a higher proportion of patients receiving oxygen therapy. Patients with PH had lower FEV 1, FVC, FEV 1/FVC, DLCO and higher serum VEGF-D levels compared with those without PH. Right heart catheterization was performed in five patients. Five patients had a pre-capillary pattern and three of these patients had severe pulmonary hypertension. Optical coherence tomography showed that there were no obvious abnormalities in the intima and lumen of the blood vessels in the two patients. Conclusions:The prevalence of PH in LAM is low. Patients in the LAM-PH group had more severe hypoxemia, worse lung function and higher serum VEGF-D levels than those in the control group. PH in LAM is mainly pre-capillary. Optical coherence tomography can evaluate the pulmonary artery vascular condition and help to identify the cause of pulmonary hypertension in LAM patients.