Journal of Pediatric surgery

Research Article|Articles in Press

Pulmonary artery measurements as postnatal prognostic tool in right congenital

diaphragmatic hernia

• Sung Hyeon Park, MD

• Ji Yoo Kim, MD
• Keon Hee Seol, MD
• Jung-Man Namgoong, MD, PhD
• Byong Sop Lee, MD, PhD
• Euiseok Jung, MD, PhD
• Show all authors

Published:December 14, 2023DOI:https://doi.org/10.1016/j.jpedsurg.2023.12.014

What is currently known about this topic?

Currently, there are no conclusive prognostic factors for right-sided congenital diaphragmatic hernia
(RCDH), particularly those assessed postnatally.

What new information is contained in this article?

This article provides novel insights into the postnatal prognostication of neonates with RCDH using the
pulmonary artery diameter. This introduces, the left PA: main PA ratio, as a robust predictor of poor
outcome.

Abstract
Background
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.
Methods
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.
Results
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
PaCO2 were associated with adverse outcomes. Notably, the LPA:MPA ratio showed the highest
predictive capability (area under the curve, 0.983; p < 0.001).
Conclusion
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 PaCO2 are
significantly correlated with outcomes.
Level of evidence
This is a level III prognostic study.