Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Republic of Korea.
Department of Pediatric Surgery, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Republic of Korea.
J Pediatr Surg. 2024 Jun;59(6):1077-1082. doi: 10.1016/j.jpedsurg.2023.12.014. Epub 2023 Dec 14.
Right-sided congenital diaphragmatic hernia (RCDH) is a rare and often fatal congenital anomaly, primarily attributed to lung hypoplasia, which is associated with small branch pulmonary artery (PA). This study investigated whether postnatal PA measurements obtained through echocardiography are associated with mortality or the extracorporeal membrane oxygenation (ECMO) requirement in neonates with RCDH.
A retrospective study was conducted on neonates with RCDH born between 2008 and 2022. Echocardiography was performed on the day of birth. The diameter of the main PA (MPA) was measured at the maximal dimension, and the diameters of the left PA (LPA) and right PA (RPA) were measured at the bifurcation. The primary outcome was mortality or ECMO requirement. Parameters, including the LPA:MPA ratio, RPA:MPA ratio, Nakata index, McGoon ratio, and ejection fraction (EF), were analyzed and compared with the observed-to-expected lung-to-head ratio (o/e LHR), initial blood gas, and defect size as predictive values.
Among 39 neonates with RCDH, 25 (64.1 %) survived without ECMO. The non-survivor or ECMO group exhibited lower o/e LHR, reduced EF, smaller LPA and RPA diameters, and larger MPA diameter than survivors. Lower LPA:MPA ratio, Nakata index, McGoon ratio, and higher initial PaCO were associated with adverse outcomes. Notably, the LPA:MPA ratio showed the highest predictive capability (area under the curve, 0.983; p < 0.001).
The LPA:MPA ratio is a promising postnatal predictor of mortality or ECMO requirement in neonates with RCDH. Additionally, Nakata index, McGoon ratio, and initial PaCO are significantly correlated with outcomes.
This is a level III.
Prognostic study.
右侧先天性膈疝（RCDH）是一种罕见且常致命的先天性异常，主要归因于肺发育不全，这与小分支肺动脉（PA）有关。本研究旨在探讨 RCDH 新生儿通过超声心动图获得的出生后 PA 测量值是否与死亡率或体外膜肺氧合（ECMO）需求相关。
对 2008 年至 2022 年期间出生的 RCDH 新生儿进行回顾性研究。在出生当天进行超声心动图检查。在最大维度测量主肺动脉（MPA）直径，在分叉处测量左肺动脉（LPA）和右肺动脉（RPA）直径。主要结局为死亡率或 ECMO 需求。分析并比较 LPA:MPA 比、RPA:MPA 比、Nakata 指数、McGoon 比和射血分数（EF）等参数与观察到的与预期的肺与头比（o/e LHR）、初始血气和缺陷大小作为预测值。
在 39 例 RCDH 新生儿中，25 例（64.1%）无 ECMO 存活。非存活或 ECMO 组的 o/e LHR 较低，EF 降低，LPA 和 RPA 直径较小，MPA 直径较大。较低的 LPA:MPA 比、Nakata 指数、McGoon 比和较高的初始 PaCO 与不良结局相关。值得注意的是，LPA:MPA 比显示出最高的预测能力（曲线下面积，0.983；p<0.001）。
LPA:MPA 比是预测 RCDH 新生儿死亡率或 ECMO 需求的有前途的出生后指标。此外，Nakata 指数、McGoon 比和初始 PaCO 与结局显著相关。
这是 III 级研究。
预后研究。
