Pulmonary hypertension associated with acute or chronic lung diseases in the preterm and term neonate and infant. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK
Pulmonary hypertension associated with acute or chronic lung diseases in the preterm and term neonate and infant. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK
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DOI：
10.1136/heartjnl-2015-308591
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发表时间：
2016-05-01
期刊：
影响因子：
5.7
通讯作者：
Hansmann, Georg
中科院分区：
文献类型：
--
作者：
Hilgendorff, Anne;Apitz, Christian;Hansmann, Georg
新生儿持续性肺动脉高压（PPHN）是最常见的新生儿形式，大多数在几天后随着潜在肺部状况的改善而可逆。当肺动脉高压（PH）在适当治疗后仍然存在时，应通过胸部CT评估肺实质疾病的严重程度。肺静脉狭窄可能需要通过心导管检查和肺活检来排除，当怀疑肺泡毛细血管发育不良时，需要进行遗传检查。在PPHN中，包括表面活性剂治疗在内的心肺状况优化应以生产SpO(2)在91%至95%之间为目标，没有三尖瓣后无限制性分流术的重症患者可接受前列腺素E1以维持右心衰时导管通畅。吸入性一氧化氮适用于机械通气婴儿，以减少体外膜氧合（ECMO）的需要，当这种治疗不可用时可考虑使用西地那非。ECMO可根据ELSO指南进行。在较大的早产儿中，PH主要与支气管肺发育不良（BPD）相关，或足月婴儿患有发育性肺异常，如先天性膈疝或心脏异常、左室舒张功能障碍/左心房高压或肺静脉狭窄，可增加疾病的复杂性。此处，口服或静脉注射西地那非应考虑用于BPD患者的PH治疗，后者用于危重患者。此外，前列腺素，矿化皮质激素受体拮抗剂和利尿剂可能是有益的。经证实或疑似PH的婴儿应接受密切随访，包括产前/导管后SpO2测量、超声心动图和包括NT-proBNP在内的实验室检查，以临床改善或缺乏临床改善为指导。
Persistent pulmonary hypertension of the newborn (PPHN) is the most common neonatal form and mostly reversible after a few days with improvement of the underlying pulmonary condition. When pulmonary hypertension (PH) persists despite adequate treatment, the severity of parenchymal lung disease should be assessed by chest CT. Pulmonary vein stenosis may need to be ruled out by cardiac catheterisation and lung biopsy, and genetic workup is necessary when alveolar capillary dysplasia is suspected. In PPHN, optimisation of the cardiopulmonary situation including surfactant therapy should aim for preductal SpO(2) between 91% and 95% and severe cases without post-tricuspid-unrestrictive shunt may receive prostaglandin E1 to maintain ductal patency in right heart failure. Inhaled nitric oxide is indicated in mechanically ventilated infants to reduce the need for extracorporal membrane oxygenation (ECMO), and sildenafil can be considered when this therapy is not available. ECMO may be indicated according to the ELSO guidelines. In older preterm infant, where PH is mainly associated with bronchopulmonary dysplasia (BPD) or in term infants with developmental lung anomalies such as congenital diaphragmatic hernia or cardiac anomalies, left ventricular diastolic dysfunction/left atrial hypertension or pulmonary vein stenosis, can add to the complexity of the disease. Here, oral or intravenous sildenafil should be considered for PH treatment in BPD, the latter for critically ill patients. Furthermore, prostanoids, mineralcorticoid receptor antagonists, and diuretics can be beneficial. Infants with proven or suspected PH should receive close follow-up, including preductal/postductal SpO2 measurements, echocardiography and laboratory work-up including NT-proBNP, guided by clinical improvement or lack thereof.