Department of Pediatrics, The Pediatric Heart Lung Center, Children's Hospital Colorado, University of Colorado Anschutz Medical Center, Aurora, Colorado, USA.
Section of Neonatology, Department of Pediatrics, Children's Hospital Colorado, University of Colorado Anschutz Medical Center, Aurora, Colorado, USA.
Pediatr Pulmonol. 2021 Mar;56(3):661-669. doi: 10.1002/ppul.25073.
Persistent pulmonary hypertension of the newborn (PPHN) is a significant clinical problem characterized by refractory and severe hypoxemia secondary to elevated pulmonary vascular resistance resulting in right-to-left extrapulmonary shunting of deoxygenated blood. PPHN is associated with diverse cardiopulmonary disorders and a high early mortality rate for infants with severe PPHN. Surviving infants with PPHN have an increased risk of long-term morbidities. PPHN physiology can be categorized by (1) maladaptation: pulmonary vessels have normal structure and number but have abnormal vasoreactivity; (2) excessive muscularization: increased smooth muscle cell thickness and increased distal extension of muscle to vessels that are usually not muscularized; and (3) underdevelopment: lung hypoplasia associated with decreased pulmonary artery number. Treatment involves adequate lung recruitment, optimization of cardiac output and left ventricular function, and pulmonary vasodilators such as inhaled nitric oxide. Infants who fail to respond to conventional therapy should be evaluated for lethal lung disorders including alveolar-capillary dysplasia, T-box transcription factor 4 gene, thyroid transcription factor-1, ATP-binding cassette A3 gene, and surfactant protein diseases.
新生儿持续性肺动脉高压（PPHN）是一种严重的临床问题，其特征为肺血管阻力升高导致难治性和严重低氧血症，从而引起脱氧血液从右向左肺外分流。PPHN 与多种心肺疾病有关，严重 PPHN 婴儿的早期死亡率较高。存活的 PPHN 婴儿有发生长期并发症的风险增加。PPHN 的生理学可以分为以下几类：（1）适应不良：肺血管具有正常的结构和数量，但血管反应性异常；（2）过度肌化：平滑肌细胞厚度增加，肌肉向通常不肌化的血管延伸增加；（3）发育不良：与肺动脉数量减少相关的肺发育不全。治疗包括充分的肺复张、优化心输出量和左心室功能，以及使用吸入性一氧化氮等肺血管扩张剂。对常规治疗无反应的婴儿应评估致死性肺部疾病，包括肺泡毛细血管发育不良、T 盒转录因子 4 基因、甲状腺转录因子-1、ATP 结合盒 A3 基因和表面活性剂蛋白疾病。
