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Clinical Characteristics and Risk Factors for Developing Pulmonary Hypertension in Children with Down Syndrome.唐氏综合征儿童发生肺动脉高压的临床特征及危险因素。
J Pediatr. 2018 Nov;202:212-219.e2. doi: 10.1016/j.jpeds.2018.06.031. Epub 2018 Jul 17.
2
Pulmonary Vein Stenosis in Infants: A Systematic Review, Meta-Analysis, and Meta-Regression.婴儿肺静脉狭窄:系统评价、荟萃分析和荟萃回归。
J Pediatr. 2018 Jul;198:36-45.e3. doi: 10.1016/j.jpeds.2018.02.030. Epub 2018 Apr 9.
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Infants Born with Down Syndrome: Burden of Disease in the Early Neonatal Period.唐氏综合征患儿:新生儿早期的疾病负担。
J Pediatr. 2018 Feb;193:21-26. doi: 10.1016/j.jpeds.2017.09.046. Epub 2017 Nov 23.
4
Pulmonary vein stenosis in patients with Smith-Lemli-Opitz syndrome.史密斯-勒米-奥皮茨综合征患者的肺静脉狭窄
Congenit Heart Dis. 2017 Jul;12(4):475-483. doi: 10.1111/chd.12471. Epub 2017 Jul 18.
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Primary Pulmonary Vein Stenosis: Outcomes, Risk Factors, and Severity Score in a Multicentric Study.原发性肺静脉狭窄:一项多中心研究的结果、危险因素及严重程度评分
Ann Thorac Surg. 2017 Jul;104(1):182-189. doi: 10.1016/j.athoracsur.2017.03.022.
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Pulmonary vein stenosis with Down syndrome: a rare and frequently fatal cause of pulmonary hypertension in infants and children.唐氏综合征合并肺静脉狭窄:婴幼儿肺动脉高压的一种罕见且常致命的病因
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Surgical management of complete atrioventricular septal defect: associations with surgical technique, age, and trisomy 21.完全性房室间隔缺损的手术治疗:与手术技术、年龄和 21 三体的相关性。
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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.

DOI:10.3390/children8010019
PMID:33401418
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7823486/
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。