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与支气管肺发育不良相关肺动脉高压患儿肺血管扩张剂治疗停药相关的因素。

Factors associated with discontinuation of pulmonary vasodilator therapy in children with bronchopulmonary dysplasia-associated pulmonary hypertension.

机构信息

Division of Cardiology, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.

Echocardiography Laboratory Research Unit, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.

出版信息

J Perinatol. 2022 Sep;42(9):1246-1254. doi: 10.1038/s41372-022-01421-6. Epub 2022 Jun 8.

Abstract

OBJECTIVE

To evaluate factors associated with discontinuation of pulmonary vasodilator therapy in bronchopulmonary dysplasia-related pulmonary hypertension (BPD-PH).

STUDY DESIGN

Retrospective study of neonatal, echocardiographic, and cardiac catheterization data in 121 infants with BPD-PH discharged on pulmonary vasodilator therapy from 2009-2020 and followed into childhood.

RESULT

After median 4.4 years, medications were discontinued in 58%. Those in whom medications were discontinued had fewer days of invasive support, less severe BPD, lower incidence of PDA closure or cardiac catheterization, and higher incidence of fundoplication or tracheostomy decannulation (p < 0.05). On multivariable analysis, likelihood of medication discontinuation was lower with longer period of invasive respiratory support [HR 0.95 (CI:0.91-0.99), p = 0.01] and worse RV dilation on pre-discharge echocardiogram [HR 0.13 (CI:0.03-0.70), p = 0.017]. In those with tracheostomy, likelihood of medication discontinuation was higher with decannulation [HR 10.78 (CI:1.98-58.59), p < 0.001].

CONCLUSION

In BPD-PH, childhood discontinuation of pulmonary vasodilator therapy is associated with markers of disease severity.

摘要

目的

评估与支气管肺发育不良相关肺动脉高压(BPD-PH)中肺血管扩张剂治疗停药相关的因素。

研究设计

对 2009 年至 2020 年期间接受肺血管扩张剂治疗并出院的 121 例 BPD-PH 婴儿的新生儿、超声心动图和心导管检查数据进行回顾性研究,并在儿童期进行随访。

结果

中位随访 4.4 年后,58%的患者停止了药物治疗。停止药物治疗的患者接受有创支持的天数较少,BPD 程度较轻,PDA 关闭或心导管检查的发生率较低,而胃底折叠术或气管切开管拔管的发生率较高(p<0.05)。多变量分析显示,有创呼吸支持时间较长[HR 0.95(CI:0.91-0.99),p=0.01]和出院前超声心动图显示 RV 扩张程度更差[HR 0.13(CI:0.03-0.70),p=0.017]与药物停药的可能性较低相关。对于接受气管切开术的患者,拔管后药物停药的可能性更高[HR 10.78(CI:1.98-58.59),p<0.001]。

结论

在 BPD-PH 中,儿童期停止肺血管扩张剂治疗与疾病严重程度的标志物相关。

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