欧洲呼吸杂志JO Eur Respir J FD欧洲呼吸学会SP PA3523 DO 10.118.3 /1393003.大会-2017。188bet官网地址PA3523 VO 50 IS supp61 A1 Mercurio, Valentina A1 Peloquin, Grace A1 Bourji, Khalil A1 Enobun, Blessing A1 Housten, Traci A1 Kolb, Todd A1 Damico, Rachel A1 Mathai, Stephen A1 Hassoun, Paul YR 2017 UL //www.qdcxjkg.com/content/50/suppl_61/PA3523.abstract AB背景:肺动脉高压(PAH)患者室性上心律失常(SVA)的发生可导致临床恶化并影响整体预后。目的:评估特发性(IPAH)和系统性硬化相关PAH (SScPAH)的SVA发生率，评估危险因素、管理和对死亡率的影响。方法:我们收集了2000年1月至2016年7月Johns Hopkins肺动脉高压登记处前瞻性登记的连续IPAH或SScPAH患者的基线人口统计学、血流动力学、6分钟步行距离和NTproBNP。在随访中记录SVA的发生情况、治疗方法和长期预后。结果:317例患者(SScPAH 201例，IPAH 116例，平均随访67±51个月)中有42例SVA(房颤34例，房扑18例，房异位性心动过速4例)。SScPAH患者的发病率略高(13.9 vs 12.1%， p=0.22)。64.3%的病例至少发生了二次发作。大多数SVA与住院相关(90.5%)。大多数病例都尝试了电复律或药理学复律。 Patients with SVA had higher right atrial pressure, pulmonary wedge pressure, NTproBNP (p<0.05) and higher prevalence of thyroid disease (69 vs 42.5%, p=0.001). Higher mortality was observed in patients with SVA (p=0.045). Recurrent SVA had higher mortality compared to single SVA episode (p=0.002). IPAH with SVA and SScPAH without SVA had a similar long term mortality (Log Rank p 0.098). SScPAH with SVA had the worst prognosis.Conclusion: SVA occurrence in PAH is a matter of concern, acutely leading to clinical deterioration and ultimately impacting long-term prognosis. Therapeutic strategies aimed at preventing SVA recurrence may be a relevant goal.