Department of Pediatrics, Children's Hospital of Philadelphia and Perelman School of Medicine, Philadelphia, PA.
Department of Neonatology, Royal Hospital for Children, Glasgow, United Kingdom.
Neoreviews. 2023 Nov 1;24(11):e720-e732. doi: 10.1542/neo.24-11-e720.
Congenital diaphragmatic hernia (CDH) results in abdominal contents entering the thoracic cavity, affecting both cardiac and pulmonary development. Maldevelopment of the pulmonary vasculature occurs within both the ipsilateral lung and the contralateral lung. The resultant bilateral pulmonary hypoplasia and associated pulmonary hypertension are important components of the pathophysiology of this disease that affect outcomes. Despite prenatal referral to specialized high-volume centers, advanced ventilation strategies, pulmonary hypertension management, and the option of extracorporeal membrane oxygenation, overall CDH mortality remains between 25% and 30%. With increasing recognition that cardiac dysfunction plays a large role in morbidity and mortality in patients with CDH, it becomes imperative to understand the different clinical phenotypes, thus allowing for individual patient-directed therapies. Further research into therapeutic interventions that address the cardiopulmonary interactions in patients with CDH may lead to improved morbidity and mortality outcomes.
先天性膈疝 (CDH) 导致腹部内容物进入胸腔，影响心脏和肺部的发育。同侧肺和对侧肺的肺血管发育不良均会发生。由此导致的双侧肺发育不全和相关的肺动脉高压是该疾病病理生理学的重要组成部分，会影响治疗结果。尽管产前转诊至专业大容量中心、采用先进的通气策略、管理肺动脉高压以及选择体外膜氧合，但 CDH 的总体死亡率仍在 25%至 30%之间。随着人们越来越认识到心脏功能障碍在 CDH 患者的发病率和死亡率中起重要作用，因此必须了解不同的临床表型，从而为每个患者提供个体化的治疗方法。进一步研究针对 CDH 患者心肺相互作用的治疗干预措施，可能会改善发病率和死亡率结果。
