NeoAPACHE II. Relationship Between Radiographic Pulmonary Area and Pulmonary Hypertension, Mortality, and Hernia Recurrence in Newborns With CDH

Abstract

Congenital diaphragmatic hernia is a rare disease with high mortality and morbidity due to pulmonary hypoplasia and pulmonary hypertension. The aim of the study is to investigate the relationship between radiographic lung area and systolic pulmonary artery pressure (sPAP) on the first day of life, mortality, and hernia recurrence during the first year of life in infants with a congenital diaphragmatic hernia (CDH). A retrospective data collection was performed on 77 CDH newborns. Echocardiographic sPAP value, deaths, and recurrence cases were recorded. Lung area was calculated by tracing the lung's perimeter, excluding mediastinal structures, and herniated organs, on the preoperative chest X-ray performed within 24 h after birth. Logistic and linear regression analyses were performed. Deceased infants showed lower areas and higher sPAP values. One square centimeter of rising in the total, ipsilateral, and contralateral area was associated with a 22, 43, and 24% reduction in mortality risk. sPAP values showed a decreasing trend after birth, with a maximum of 1.84 mmHg reduction per unitary increment in the ipsilateral area at birth. Recurrence patients showed lower areas, with recurrence risk decreasing by 14 and 29% per unit increment of the total and ipsilateral area. In CDH patients, low lung area at birth reflects impaired lung development and defect size, being associated with increased sPAP values, mortality, and recurrence risk. Clinical Trial Registration: The manuscript is an exploratory secondary analysis of the trial registered at ClinicalTrials.gov with identifier NCT04396028.

Keywords: FETO; congenital diaphragmatic hernia; lung hypoplasia; mortality; pulmonary hypertension; radiographic lung area; recurrence of the hernia.

Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Copyright © 2021 Amodeo, Pesenti, Raffaeli, Macchini, Condò, Borzani, Persico, Fabietti, Bischetti, Colli, Ghirardello, Gangi, Colnaghi, Mosca and Cavallaro.

Figures

Figure 1

Study flowchart.

Figure 2

(A) Boxplots showing the comparison of sPAP values between survived and deceased patients. Student's t-test was performed to compare the two groups. (1) sPAP T0, p = 0.016; (2) sPAP T1, p = 0.022; (3) sPAP T2, p = 0.163; (4) sPAP T3, p < 0.001. (B) Boxplots showing the comparison of radiographic lung area on the first day of life between survived and deceased patients. Student's t-test was performed to compare the two groups. (1) Total pulmonary area, p < 0.001; (2) ipsilateral pulmonary area, p < 0.001; (3) contralateral pulmonary area, p < 0.001. (C) Boxplots showing the comparison of radiographic lung area on the first day of life between non-recurrence and recurrence patients. Student's t-test was performed to compare the two groups. (1) Total pulmonary area, p = 0.034; (2) ipsilateral pulmonary area, p = 0.011; (3) contralateral pulmonary area, p = 0.164.

Figure 3

(A) ROC analysis and estimation of the corresponding area under the curve (AUC) for the radiographic pulmonary area's performance in predicting survival to 1 year of life. (1) Total pulmonary area; (2) ipsilateral pulmonary area; (3) contralateral pulmonary area. (B) ROC analysis and estimation of the corresponding area under the curve (AUC) for the O/E LHR% at diagnosis performance in predicting hernia recurrence in the first year of life. (C) ROC analysis and estimation of the corresponding area under the curve (AUC) for the radiographic performance of the pulmonary area in predicting hernia recurrence in the first year of life. (1) Total pulmonary area; (2) ipsilateral pulmonary area.