TY - JOUR T1 - BMPR2突变患者的胸部CT特征:来自英国国家特发性和遗传性PAH队列研究的初步分析PA5454六世- 54 - 5 63 SP - PA5454盟Cogliano玛塞拉AU -洛瑞·艾伦AU -基利,大卫·g . AU - Condliffe罗宾·a . AU -拉贾拉姆Smitha盟——Screaton尼古拉斯盟——Hadinnapola查拉卡非盟- Swietlik,伊米莉亚m . AU -格拉芙,Stefan盟——霍华德、卢克·s . AU - Coghlan Gerry盟——麦芽汁、美国约翰AU - Pepke-Zaba,乔安娜盟——LordanJames AU - Johnson, Martin K. AU - Wilkins, Martin R. AU - Morrell, Nicholas W. AU - Swift, Andrew J. Y1 - 2019/09/28 UR - //www.qdcxjkg.com/content/54/suppl_63/PA5454.abstract N2 -介绍:骨形态发生受体2 (BMPR2)基因突变是遗传性肺动脉高压(PAH)最常见的遗传原因。本研究的目的是研究特发性和遗传性PAH和BMPR2突变患者的胸部CT (CT)特征。方法:选择特发性或遗传性PAH患者;排除了BMPR2以外突变的患者。在不了解BMPR2状态的观察者中评估CT特征。采用独立t检验、Chi2检验、二元logistic回归和单因素ANCOVA对BMPR2突变患者进行比较。结果:在Sheffield肺血管疾病单位111例IPAH或HPAH患者的初步分析中，19例有BMPR2突变。突变携带者更年轻(p=0.009)。 No sex difference was identified (p=0.313). New peripheral lung blood vessels ‘neovascularity’ and enlarged bronchial arteries were observed in BMPR2 mutation carriers (p<0.05). There was no difference in right ventricular (RV) size, mean pulmonary arterial pressure (mPAP) and pulmonary vascular resistance (PVR) (p>0.05). After adjusting for age, sex, body surface area, WHO functional class, mPAP and PVR, a smaller left atrial area and smaller main pulmonary artery diameter were associated with a BMPR2 mutation.Conclusion: In this preliminary analysis of the UK National IPAH cohort, we have observed that BMPR2 carriers had smaller pulmonary arteries and smaller left atria. Whether this reflects differences in RV afterload and left ventricle preload requires further investigation.FootnotesCite this article as: European Respiratory Journal 2019; 54: Suppl. 63, PA5454.This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only). ER -