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, I 2 ?=?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, I 2 ?=?24%). Furthermore, the difference was significant only in male patients (OR?=?5.58, 95% CI: 2.16~14.39, P?=?0.0004, I 2 ?=?0%) and not in female patients (OR?=?1.41, 95% CI: 0.75~2.67, P?=?0.29, I 2 ?=?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.
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