Chaudhari Tejasvi, Schmidt Sotomayor Nadia, Maheshwari Rajesh
Department of Neonatology, The Canberra Hospital, Canberra, ACT, Australia.
Australian National University Medical School, Australian National University, Canberra, ACT, Australia.
Front Pediatr. 2024 Mar 25;12:1356157. doi: 10.3389/fped.2024.1356157. eCollection 2024.
Congenital diaphragmatic hernia (CDH) is a developmental defect of the diaphragm resulting in herniation of viscera into the chest. This condition is characterized by pulmonary hypoplasia, pulmonary hypertension (PH) and cardiac ventricular dysfunction. PH is a key component of the pathophysiology of CDH in neonates and contributes to morbidity and mortality. Traditionally, PH associated with CDH (CDH-PH) is thought to be secondary to increased pulmonary arterial resistance and vasoreactivity resulting from pulmonary hypoplasia. Additionally, there is increasing recognition of associated left ventricular hypoplasia, dysfunction and elevated end diastolic pressure resulting in pulmonary venous hypertension in infants with CDH. Thus, hemodynamic management of these infants is complex and cautious use of pulmonary vasodilators such as inhaled nitric oxide (iNO) is warranted. We aim to provide an overview of different phenotypic profiles of CDH associated PH and potential management options based on current evidence and pathophysiology.
先天性膈疝（CDH）是一种膈肌发育缺陷，导致内脏疝入胸腔。这种病症的特征是肺发育不全、肺动脉高压（PH）和心室功能障碍。PH是新生儿CDH病理生理学的关键组成部分，会导致发病和死亡。传统上，与CDH相关的PH（CDH-PH）被认为是继发于肺发育不全导致的肺动脉阻力增加和血管反应性增强。此外，越来越多的人认识到，CDH婴儿存在相关的左心室发育不全、功能障碍和舒张末期压力升高，从而导致肺静脉高压。因此，这些婴儿的血流动力学管理很复杂，有必要谨慎使用吸入一氧化氮（iNO）等肺血管扩张剂。我们旨在根据当前证据和病理生理学，概述CDH相关PH的不同表型特征以及潜在的管理方案。
