Vasoreactive phenotype in children with pulmonary arterial hypertension and syncope

Author:

Linder Alexandra N.ORCID,Hsia Jill,Krishnan Sheila V.,Rosenzweig Erika B.ORCID,Krishnan Usha S.

Abstract

BackgroundSyncope in Group 1 pulmonary arterial hypertension (PAH) is an independent predictor of poor prognosis in adults, but this is not well studied in children. We hypothesise that syncope in children with PAH often occurs in association with a reactive pulmonary vascular bed with sudden vasoconstriction in response to adverse stimuli. In the current study, we sought to determine the association of syncope with acute vasoresponsiveness and outcomes in children with Group 1 PAH.MethodsA retrospective chart review of children with PAH at a single pulmonary hypertension centre from 1 January 2005 to 31 October 2018 was performed. Data included demographics, symptoms, imaging, haemodynamics, and outcomes at baseline and follow-up.Results169 children had Group 1 PAH; 47 (28%) had syncope at presentation or follow-up. Children with significant shunts were excluded from the analysis. Children with syncope were older at diagnosis (7.5versus5.0 years; p=0.002) and had a higher incidence of chest pain (p=0.022) and fatigue (p=0.003). They had higher pulmonary vascular resistance at baseline (14.9versus9.1 WU·m2; p=0.01). More children with syncope were vasoresponders to inhaled nitric oxide (33%versus22%; p=0.08–NS). Children with syncope and acute vasoresponsiveness had the highest survival, and non-responders with syncope on medications had the worst long-term survival.ConclusionsChildren with syncope had higher rates of vasoreactivity compared to those without. This suggests that in some children with PAH, syncope may simply reflect acute pulmonary vasoconstriction to an adverse stimulus. Larger prospective studies are warranted to further assess syncope as a marker for a vasoreactive phenotype with implications for treatment and long-term outcomes.

Publisher

European Respiratory Society (ERS)

Subject

Pulmonary and Respiratory Medicine

Cited by 1 articles. 订阅此论文施引文献 订阅此论文施引文献,注册后可以免费订阅5篇论文的施引文献,订阅后可以查看论文全部施引文献

同舟云学术

1.学者识别学者识别

2.学术分析学术分析

3.人才评估人才评估

"同舟云学术"是以全球学者为主线,采集、加工和组织学术论文而形成的新型学术文献查询和分析系统,可以对全球学者进行文献检索和人才价值评估。用户可以通过关注某些学科领域的顶尖人物而持续追踪该领域的学科进展和研究前沿。经过近期的数据扩容,当前同舟云学术共收录了国内外主流学术期刊6万余种,收集的期刊论文及会议论文总量共计约1.5亿篇,并以每天添加12000余篇中外论文的速度递增。我们也可以为用户提供个性化、定制化的学者数据。欢迎来电咨询!咨询电话:010-8811{复制后删除}0370

www.globalauthorid.com

TOP

Copyright © 2019-2024 北京同舟云网络信息技术有限公司
京公网安备11010802033243号  京ICP备18003416号-3