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Assessment of pulmonary artery size at birth as a prognostic factor in congenital diaphragmatic hernia: results of a multicenter study in Japan



To assess the reliability of pulmonary artery (PA) parameters as a prognostic marker in neonates with isolated left-sided congenital diaphragmatic hernia (IL-CDH).

Study design

A retrospective cohort study conducted by the Japanese CDH Study Group (JCDHSG).


323 IL-CDH patients registered with the JCDHSG were included. 272 patients survived to 90 days of age. Right PA (RPA) and left PA (LPA) diameters and pulmonary artery index (PAIndex) at birth were significantly larger in survivors. The cutoff values of RPA and LPA diameters and PAIndex for survival up to 90 days were 3.2 mm, 2.8 mm and 83.7, respectively, and logistic regression analysis showed that these were significantly related to survival. Multiple logistic regression analysis showed that both the PA parameters and liver herniation were significantly related to survival.


The three PA parameters at birth can predict clinical outcomes and are considered as independent risk factors of liver herniation.

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Fig. 1: Receiver operating characteristic (ROC) curves of the parameters.
Fig. 2: Receiver operating characteristic (ROC) curves of the combination of the parameters.

Data availability

The datasets generated and/analyzed during current study are available from the corresponding author on reasonable request.


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We would like to thank all members of JCDHSG for managements of patients and registrations to the database of JCDHSG. Funding: This study was funded by the Ministry of Health, Labour, and Welfare of Japan (Grant/Award Number: H24-Ninchi-Ippan-034).

Author information

Authors and Affiliations



TO, KT, OH, SA, and NI made substantial contributions to the study conception and design. All authors performed clinical data collection. SN, YN, and NY made substantial contributions to statistical analysis and data interpretation. TO drafted the manuscript, and KT, MH, OH, KN, and NU reviewed the manuscript and provided critical feedback. All authors approved the final manuscript as submitted and agree to be accountable for all aspects of the work.

Corresponding author

Correspondence to Tadaharu Okazaki.

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The authors declare no competing interests.

Ethical approval

The protocol of this study was approved by the Ethics Committee of Osaka University Hospital (approval number 11017) and the committees at all participating institutions. The investigation was performed in accordance with the principles of the Declaration of Helsinki and the Ethical Guidelines for Medical and Health Research Involving Human Subjects.

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Okazaki, T., Terui, K., Nagata, K. et al. Assessment of pulmonary artery size at birth as a prognostic factor in congenital diaphragmatic hernia: results of a multicenter study in Japan. J Perinatol 43, 1295–1300 (2023).

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