OBJECTIVE To investigate the differences in the proportions of BMPR2 mutations in familial hereditary pulmonary arterial hypertension (HPAH) and idiopathic pulmonary arterial hypertension (IPAH) between males and females and the relationship between BMPR2 mutation and PAH severity. METHODS A computer was used to search the electronic Cochrane Library, PubMed/MEDLINE, and EMBASE databases for clinical trials containing information on the relationship between PAH prognosis and BMPR2 mutations through March 2019. After obtaining the data, a meta-analysis was performed using Review Manager Version 5.3 and Stata. RESULTS A meta-analysis was performed on 17 clinical trials (2198 total patients: 644 male, 1554 female). The results showed that among patients with HPAH and IPAH, the BMPR2 mutation rate is higher in male than in female patients [male group (224/644, 34.78%), female group (457/1554, 29.41%), OR = 1.30, 95% CI: 1.06~1.60, P = 0.01, I2 = 10%]. Furthermore, haemodynamic and functional parameters were more severe in IPAH and HPAH patients with BMPR2 mutations than in those without, and those with BMPR2 mutation were diagnosed at a younger age. The risk of death or transplantation was higher in PAH patients with BMPR2 mutations than in those without (OR = 2.51, 95% CI: 1.29~3.57, P = 0.003, I2 = 24%). Furthermore, the difference was significant only in male patients (OR = 5.58, 95% CI: 2.16~14.39, P = 0.0004, I2 = 0%) and not in female patients (OR = 1.41, 95% CI: 0.75~2.67, P = 0.29, I2 = 0%). CONCLUSION Among patients with HPAH and IPAH, men are more likely to have BMPR2 mutations, which may predict more severe PAH indications and prognosis.

中文翻译：

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患有BMPR2突变的肺动脉高压患者的性别差异：一项荟萃分析。

目的探讨男女遗传性家族性遗传性肺动脉高压（HPAH）和特发性肺动脉高压（IPAH）中BMPR2突变比例的差异，以及BMPR2突变与PAH严重程度的关系。方法使用计算机在电子Cochrane库，PubMed / MEDLINE和EMBASE数据库中搜索临床试验，该临床试验包含截至2019年3月PAH预后与BMPR2突变之间关系的信息。获得数据后，使用Review进行荟萃分析。 Manager 5.3版和Stata。结果对17项临床试验进行了荟萃分析（总共2198例患者：644例男性，1554例女性）。结果显示，在HPAH和IPAH患者中，男性的BMPR2突变率高于女性[男性组（224/644，34.78％），女性组（457/1554，29.41％），OR = 1.30，95％CI：1.06〜1.60，P = 0.01 ，I2 = 10％]。此外，具有BMPR2突变的IPAH和HPAH患者的血流动力学和功能参数比没有BMPR2突变的患者更严重，诊断为BMPR2突变的患者年龄较小。具有BMPR2突变的PAH患者的死亡或移植风险高于没有BMPR2突变的PAH患者（OR = 2.51，95％CI：1.29〜3.57，P = 0.003，I2 = 24％）。此外，差异仅在男性患者中显着（OR = 5.58，95％CI：2.16〜14.39，P = 0.0004，I2 = 0％），而在女性患者中则无统计学意义（OR = 1.41，95％CI：0.75〜2.67， P = 0.29，I2 = 0％）。结论在HPAH和IPAH患者中，男性更可能具有BMPR2突变，