Abstract：
Congenital diaphragmatic hernia (CDH) is associated with high mortality rates and significant pulmonary morbidities. The objective of this study was to delineate the histopathological features observed in necropsies of CDH patients and correlate these with their clinical manifestations.
We retrospectively reviewed the postmortem findings and corresponding clinical characteristics in eight CDH cases from 2017 to July 2022.
The median survival time was 46 (8-624) hours. Autopsy reports showed that diffuse alveolar damage (congestion and hemorrhage) and hyaline membrane formation were the primary pathological lung changes observed. Notably, despite significant reduction in lung volume, the lung development appeared normal in 50% of the cases, while lobulated deformities were present in three (37.5%) cases. All patients displayed a large patent ductus arteriosus (PDA) and a patent foramen ovale, resulting in increased right ventricle (RV) volume, and myocardial fibers appeared slightly congested and swollen. The pulmonary vessels indicated thickening of the arterial media and adventitia. Lung hypoplasia and diffuse lung damage resulted in impaired gas exchange, while PDA and pulmonary hypertension led to RV failure, subsequent organ dysfunction and ultimately death.
Patients with CDH typically succumb to cardiopulmonary failure, a condition driven by a complex interplay of pathophysiological factors. This complexity accounts for the unpredictable response to currently available vasodilators and ventilation therapies.
We retrospectively reviewed the postmortem findings and corresponding clinical characteristics in eight CDH cases from 2017 to July 2022.
The median survival time was 46 (8-624) hours. Autopsy reports showed that diffuse alveolar damage (congestion and hemorrhage) and hyaline membrane formation were the primary pathological lung changes observed. Notably, despite significant reduction in lung volume, the lung development appeared normal in 50% of the cases, while lobulated deformities were present in three (37.5%) cases. All patients displayed a large patent ductus arteriosus (PDA) and a patent foramen ovale, resulting in increased right ventricle (RV) volume, and myocardial fibers appeared slightly congested and swollen. The pulmonary vessels indicated thickening of the arterial media and adventitia. Lung hypoplasia and diffuse lung damage resulted in impaired gas exchange, while PDA and pulmonary hypertension led to RV failure, subsequent organ dysfunction and ultimately death.
Patients with CDH typically succumb to cardiopulmonary failure, a condition driven by a complex interplay of pathophysiological factors. This complexity accounts for the unpredictable response to currently available vasodilators and ventilation therapies.
摘要：
背景：先天性膈疝（CDH）与高死亡率和显著的肺部发病率相关。这项研究的目的是描述CDH患者尸检中观察到的组织病理学特征，并将其与临床表现相关联。
方法：我们回顾性回顾了2017年至2022年7月8例CDH患者的死后发现和相应的临床特征。
结果：中位生存时间为46（8-624）小时。尸检报告显示，弥漫性肺泡损伤（充血和出血）和透明膜形成是观察到的主要病理性肺改变。值得注意的是,尽管肺体积显著减少，50%的病例肺发育正常,3例（37.5％）存在分叶状畸形。所有患者均表现为大动脉导管未闭（PDA）和卵圆孔未闭，导致右心室（RV）体积增加，心肌纤维出现轻微充血和肿胀。肺血管显示动脉中膜和外膜增厚。肺发育不全和弥漫性肺损伤导致气体交换受损，而PDA和肺动脉高压导致RV衰竭，随后的器官功能障碍和最终死亡。
结论：CDH患者通常会死于心肺衰竭，由病理生理因素的复杂相互作用驱动的状况。这种复杂性解释了对当前可用的血管扩张剂和通气疗法的不可预测的反应。
方法：我们回顾性回顾了2017年至2022年7月8例CDH患者的死后发现和相应的临床特征。
结果：中位生存时间为46（8-624）小时。尸检报告显示，弥漫性肺泡损伤（充血和出血）和透明膜形成是观察到的主要病理性肺改变。值得注意的是,尽管肺体积显著减少，50%的病例肺发育正常,3例（37.5％）存在分叶状畸形。所有患者均表现为大动脉导管未闭（PDA）和卵圆孔未闭，导致右心室（RV）体积增加，心肌纤维出现轻微充血和肿胀。肺血管显示动脉中膜和外膜增厚。肺发育不全和弥漫性肺损伤导致气体交换受损，而PDA和肺动脉高压导致RV衰竭，随后的器官功能障碍和最终死亡。
结论：CDH患者通常会死于心肺衰竭，由病理生理因素的复杂相互作用驱动的状况。这种复杂性解释了对当前可用的血管扩张剂和通气疗法的不可预测的反应。
