Congenital diaphragmatic hernia (CDH) is a rare developmental defect of the diaphragm, causing considerable morbidity and mortality1,2. Morphological changes are present from an early developmental stage, consisting of increased muscularization of the pulmonary vessels, rarefication of pulmonary arterioles and capillaries, and a decreased alveolar density3. Consecutively, most CDH newborns present with pulmonary hypertension (PH) and lung hypoplasia which are associated with mortality4. The severity of PH and lung hypoplasia may subsequently contribute through several mechanisms, such as mechanical ventilation-induced lung damage, to long-term oxygen dependency and the development of chronic lung disease (CLD), which can be defined as oxygen dependency at 28 days of life. The cellular and molecular mechanisms initiating and contributing to PH and CLD are only partially understood5. However, morphological changes are likely being enhanced in response to hypoxia, inflammation, oxidative stress and injury of the bronchoalveolar system and the pulmonary vessels6.
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