Treprostinil in Neonates with Congenital Diaphragmatic Hernia-Related Pulmonary Hypertension.
Treprostinil in Neonates with Congenital Diaphragmatic Hernia-Related Pulmonary Hypertension.
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曲前列环素用于患有先天性膈疝相关肺动脉高压的新生儿。
DOI：
10.1016/j.jpeds.2023.113420
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发表时间：
2023
期刊：
影响因子：
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通讯作者：
Hedrick,HollyL
中科院分区：
文献类型：
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作者：
DeBie,FelixR;Avitabile,CatherineM;Flohr,Sabrina;Land,Sierra;Mathew,Leny;Wang,Yan;Ash,Devon;Rintoul,NatalieE;Hedrick,HollyL
目的描述我们的经验与曲前列尼尔，评估与心脏功能的相关性，并评估先天性疝相关肺动脉高压（CDH-PH）的新生儿的不良反应。研究设计一个回顾性审查的单中心前瞻性登记在四级护理儿童医院。纳入研究的患者在2013年4月至2021年9月期间接受了曲前列尼尔治疗的CDH-PH。评估的结果是在基线、曲前列环素治疗开始后1周、2周和1个月收集的脑型利钠肽水平和定量超声心动图参数。通过三尖瓣环平面收缩期偏移Z评分和斑点追踪超声心动图（整体纵向和游离壁应变）评估右心室（RV）功能。间隔位置和左心室（LV）压缩偏心指数和M-模式Z-scores.ResultsFifty-one患者进行了评估，平均预期/观察肺头比为28.4 ± 9.0%。大多数患者需要体外膜肺氧合（n = 45，88%）。出院存活率为31/49（63%）。曲前列环素在中位年龄为19天时开始，中位有效剂量为34 ng/kg/min。1个月后，中位基线脑型利钠肽水平从416.9 pg/mL降至120.5 pg/mL。曲前列尼尔与改善的三尖瓣环平面收缩期偏移Z评分、RV整体纵向应变、RV游离壁应变、LV偏心指数以及LV舒张期和收缩期尺寸相关，反映了RV的压缩较小，无论最终患者生存率如何。没有严重的不良反应recorded.ConclusionsIn新生儿CDH-PH，曲前列尼尔管理是耐受性良好，并与改善RV的大小和功能。
ObjectiveTo describe our experience with treprostinil, evaluate correlations with cardiac function, and assess for adverse effects in neonates with congenital diaphragmatic hernia-related pulmonary hypertension (CDH-PH).Study designA retrospective review of a single-center prospective registry at a quaternary care children's hospital. Patients included in the study had CDH-PH treated with treprostinil between April 2013 and September 2021. Assessed outcomes were brain-type natriuretic peptide levels and quantitative echocardiographic parameters collected at baseline, 1 week, 2 weeks, and 1 month after treprostinil initiation. Right ventricular (RV) function was assessed by tricuspid annular plane systolic excursion Z-score and speckle tracking echocardiography (global longitudinal and free wall strain). Septal position and left ventricular (LV) compression were assessed by eccentricity index and M-mode Z-scores.ResultsFifty-one patients were included, with an average expected/observed lung-to-head ratio of 28.4 ± 9.0%. Most patients required extra-corporeal membrane oxygenation (n = 45, 88%). Survival to hospital discharge was 31/49 (63%). Treprostinil was initiated at a median age of 19 days with a median effective dose of 34 ng/kg/minute. Median baseline brain-type natriuretic peptide level decreased from 416.9 pg/mL to 120.5 pg/mL after 1 month. Treprostinil was associated with improved tricuspid annular plane systolic excursion Z-score, RV global longitudinal strain, RV free wall strain, LV eccentricity index, and LV diastolic and systolic dimensions, reflecting less compression by the RV, regardless of ultimate patient survival. No serious adverse effects were recorded.ConclusionsIn neonates with CDH-PH, treprostinil administration is well tolerated and is associated with improved RV size and function.