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长时间暴露于左向右分流是21三体综合征患者发生肺静脉狭窄的一个危险因素。

Longer Exposure to Left-to-Right Shunts Is a Risk Factor for Pulmonary Vein Stenosis in Patients with Trisomy 21.

作者信息

Choi Connie, Gauvreau Kimberlee, Levy Philip, Callahan Ryan, Jenkins Kathy J, Chen Minghui

机构信息

Department of Cardiology, Harvard Medical School, Boston Children's Hospital, Boston, MA 02115, USA.

Division of Newborn Medicine, Department of Pediatrics, Harvard Medical School, Boston Children's Hospital, Boston, MA 02115, USA.

出版信息

Children (Basel). 2021 Jan 1;8(1):19. doi: 10.3390/children8010019.

Abstract

We conducted a study to determine whether patients born with Trisomy 21 and left-to-right shunts who develop pulmonary vein stenosis (PVS) have a longer exposure to shunt physiology compared to those who do not develop PVS. We included patients seen at Boston Children's Hospital between 15 August 2006 and 31 August 2017 born with Trisomy 21 and left-to-right shunts who developed PVS within 24 months of age. We conducted a retrospective 3:1 matched case-control study. The primary predictor was length of exposure to shunt as defined as date of birth to the first echocardiogram showing mild or no shunt. Case patients with PVS were more likely to have a longer exposure to shunt than patients in the control group (6 vs. 3 months, -value 0.002). Additionally, PVS patients were also more likely to have their initial repair ≥ 4 months of age (81% vs. 42%, -value 0.003) and have a gestational age ≤ 35 weeks (48% vs. 13%, -value 0.003). Time exposed to shunts may be an important modifiable risk factor for PVS in patients with Trisomy 21.

摘要

我们开展了一项研究,以确定患有21三体综合征且存在左向右分流并发生肺静脉狭窄(PVS)的患者,与未发生PVS的患者相比,是否有更长时间处于分流生理状态。我们纳入了2006年8月15日至2017年8月31日期间在波士顿儿童医院就诊的、患有21三体综合征且存在左向右分流并在24个月龄内发生PVS的患者。我们进行了一项回顾性3:1匹配病例对照研究。主要预测因素是分流暴露时长,定义为从出生到首次超声心动图显示轻度或无分流的日期。与对照组患者相比,患有PVS的病例患者更有可能有更长的分流暴露时间(6个月对3个月,P值0.002)。此外,PVS患者也更有可能在≥4个月龄时进行初次修复(81%对42%,P值0.003),且胎龄≤35周(48%对13%,P值0.003)。对于21三体综合征患者,分流暴露时间可能是PVS一个重要的可改变风险因素。

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