Pediatric Pulmonary Hypertension: Definitions, Mechanisms, Diagnosis, and Treatment.
Pediatric Pulmonary Hypertension: Definitions, Mechanisms, Diagnosis, and Treatment.
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DOI：
10.1002/cphy.c200023
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发表时间：
2021-06-30
影响因子：
5.8
通讯作者：
Konduri GG
中科院分区：
文献类型：
--
作者：
Mukherjee D;Konduri GG
小儿肺动脉高压（PPH）是一种多因素疾病，具有不同的病因和表现特征。肺动脉高压（PH）是指肺动脉压力升高，是多种肺血管疾病的主要表现。它通常是其他肺部疾病的隐藏成分，如囊性纤维化和支气管肺发育不良。肺发育和遗传条件的改变是儿科肺动脉高压疾病的重要因素，这是一个不同于成人PH的实体。许多儿科PH的原因都是产前发病，由于母体和胎儿的条件改变了肺发育。由于肺生长在导致PPH的几种情况下发生改变，PPH的治疗包括肺血管扩张剂和恢复肺生长的策略。这些策略包括最佳肺泡复张、维持生理血气张力、营养支持和解决影响因素，如气道疾病和胃食管反流。PH婴儿和儿童的结局具有高度可变性，并且在很大程度上取决于根本原因。最好的结果是患有持续性肺动脉高压（PPHN）和可逆性肺部疾病的新生儿，而一些遗传性疾病（例如肺泡毛细血管发育不良）是致命的。
Pediatric pulmonary hypertension (PPH) is a multifactorial disease with diverse etiologies and presenting features. Pulmonary hypertension (PH), defined as elevated pulmonary artery pressure, is the presenting feature for several pulmonary vascular diseases. It is often a hidden component of other lung diseases, such as cystic fibrosis and bronchopulmonary dysplasia. Alterations in lung development and genetic conditions are an important contributor to pediatric pulmonary hypertensive disease, which is a distinct entity from adult PH. Many of the causes of pediatric PH have prenatal onset with altered lung development due to maternal and fetal conditions. Since lung growth is altered in several conditions that lead to PPH, therapy for PPH includes both pulmonary vasodilators and strategies to restore lung growth. These strategies include optimal alveolar recruitment, maintaining physiologic blood gas tension, nutritional support, and addressing contributing factors, such as airway disease and gastroesophageal reflux. The outcome for infants and children with PH is highly variable and largely dependent on the underlying cause. The best outcomes are for neonates with persistent pulmonary hypertension (PPHN) and reversible lung diseases, while some genetic conditions such as alveolar capillary dysplasia are lethal.
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