Primary pulmonary vein stenosis among premature infants with single-vessel disease.

Primary pulmonary vein stenosis among premature infants with single-vessel disease.
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DOI:
10.1038/s41372-020-00830-9
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发表时间:
2021-07
期刊:
Journal of perinatology : official journal of the California Perinatal Association
影响因子:
--
通讯作者:
Backes CH
Backes CH
中科院分区:
其他
文献类型:
--
作者:
Zettler E;Rivera BK;Stiver C;Boe B;Cua C;Ball MK;Smith CV;Slaughter JL;Chen B;Callahan R;Backes CH

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描述诊断为单支原发性肺静脉狭窄(PPVS)的早产儿最初采用保守治疗(主动监测并延迟治疗)的结局。一项在妊娠<37周的婴儿中进行的单血管PPVS回顾性队列研究(2009-2019年)。婴儿分为2类:疾病进展和疾病稳定。检查心脏病结局,并进行Kaplan-Meier生存分析。其中包括20名婴儿。与稳定组相比(0/10,0%),进展组所有婴儿至少有重度狭窄或闭锁(10/10,100%; P<0.01)。诊断时重度肺动脉高压在进展组(5/10,50%)比稳定组(0/10,0%; P=0.03)增加。进展组婴儿的存活率低于稳定组(Log-Rank检验,P<0.01)。在患有单血管PPVS的早产儿中,可以进行风险分层,其中可以应用更有针对性的个体化治疗。
Describe outcomes among preterm infants diagnosed with single-vessel primary pulmonary vein stenosis (PPVS) initially treated using conservative management (active surveillance with deferral of treatment). Retrospective cohort study at a single, tertiary-center (2009-2019) among infants <37 weeks’ gestation with single-vessel PPVS. Infants were classified into 2 categories: disease progression and disease stabilization. Cardiopulmonary outcomes were examined, and a Kaplan-Meier survival analysis performed. Twenty infants were included. Compared to infants in the stable group (0/10, 0%), all infants in the progressive group had development of at least severe stenosis or atresia (10/10, 100%; P<0.01). Severe pulmonary hypertension at diagnosis was increased in the progressive (5/10, 50%) than the stable group (0/10, 0%; P=0.03). Survival was lower among infants in the progressive than the stable group (Log-Rank Test, P<0.01). Among preterm infants with single-vessel PPVS, risk stratification may be possible, wherein more targeted, individualized therapies could be applied.
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