Carpentier E, Mur S, Aubry E, Pognon L, Rakza T, Flamein F, Sharma D, Tourneux P, Storme L
Department of Neonatology, University Hospital of Lille, 2 Avenue Oscar Lambret, 59037 Lille, France,; Department of Neonatology, University Hospital of Amiens, Place Laennec, 80054 Amiens, Cédex 1, France; PériTox Laboratory (UMI- 01), University of Amiens, UPJV, Place Laennec, 80054 Amiens, Cédex 1, France.
Department of Neonatology, University Hospital of Lille, 2 Avenue Oscar Lambret, 59037 Lille, France.
J Pediatr Surg. 2017 Sep;52(9):1480-1483. doi: 10.1016/j.jpedsurg.2017.03.058. Epub 2017 Mar 28.
Prolonged pulmonary hypertension (PH) is highly predictive for pulmonary morbidity and death in infants with congenital diaphragmatic hernia (CDH).
To report the effects and tolerability of subcutaneous treprostinil in newborns with severe CDH and late life-threatening PH.
We recorded clinical and echocardiography data before and after starting subcutaneous treprostinil, on patients with severe CDH and late PH, refractory to inhaled nitric oxide and oral sildenafil.
14 patients were treated with treprostinil (gestational age: 39.1±2.0weeks; birth weight: 3200±600g). Prior to treatment, the pre- and post-ductal SpO2 difference (Δ SpO2) was 14±10%. Treprostinil was initiated at a median age of 12days [5-157]. After starting treprostinil, ΔSpO2 decreased to 3% at day 7 (p<0.05), and the mean blood flow velocities in the right pulmonary arteries increased by 110% (p<0.05). 2 of the 14 patients died. At the age of follow up (12months to 3years), the 12 surviving infants were all weaned from respiratory support and discharged home.
The subcutaneous treprostinil improves pulmonary hemodynamics and outcomes in infants with CDH and life-threatening PH. We suggest that the treatment should be considered in infants with severe CDH and late PH.
Case series with no comparison group.
Level IV.
先天性膈疝（CDH）患儿的长期肺动脉高压（PH）对肺部发病和死亡具有高度预测性。
报告皮下注射曲前列尼尔对患有严重CDH和晚期危及生命的PH的新生儿的疗效和耐受性。
我们记录了患有严重CDH和晚期PH且对吸入一氧化氮和口服西地那非难治的患者开始皮下注射曲前列尼尔前后的临床和超声心动图数据。
14例患者接受了曲前列尼尔治疗（胎龄：39.1±2.0周；出生体重：3200±600g）。治疗前，导管前和导管后血氧饱和度差异（ΔSpO2）为14±10%。曲前列尼尔开始使用的中位年龄为12天[5 - 157]。开始使用曲前列尼尔后，第7天ΔSpO2降至3%（p<0.05），右肺动脉平均血流速度增加了110%（p<0.05）。14例患者中有2例死亡。在随访年龄（12个月至3年）时，12名存活婴儿均脱离呼吸支持并出院回家。
皮下注射曲前列尼尔可改善患有CDH和危及生命的PH的婴儿的肺血流动力学和预后。我们建议对患有严重CDH和晚期PH的婴儿考虑进行该治疗。
无比较组的病例系列。
IV级。
