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. 2023 Aug 30;12(8):1490-1503.
doi: 10.21037/tp-23-14. Epub 2023 Jul 26.

Pulmonary outcomes of congenital diaphragmatic hernia patients based on defect size (CDH Study Group Stage)

Hina Emanuel  1 , Hannah V Breitschopf  2 , Matthew T Harting  2 , Diana J Martinez Castillo  1 , Aravind Yadav  1 , Katrina McBeth  1 , S Syed Hashmi  1 , Ashley H Ebanks  2 , Tomika S Harris  1 , Kevin P Lally  2 , Cindy K Jon  1 , James M Stark  1 , Ricardo A Mosquera  1
Affiliations

Pulmonary outcomes of congenital diaphragmatic hernia patients based on defect size (CDH Study Group Stage)

Hina Emanuel et al. Transl Pediatr. .

Abstract

Background: Congenital diaphragmatic hernia (CDH) is associated with significant pulmonary morbidity. Previous investigation has shown that postnatal inpatient morbidity is linked to diaphragmatic defect size. The objective of this study was to evaluate long-term pulmonary outcomes by CDH study group defect size.

Methods: A retrospective analysis was conducted for CDH patients (n=133) managed in a neonatal intensive care unit (NICU) at a single children's hospital within an adult hospital system and subsequently followed up at a comprehensive multidisciplinary CDH clinic (n=102) from January 2012 to April 2022. CDH patients were stratified according to Congenital Diaphragmatic Hernia Study Group (CDHSG) Stage, and then categorized as low-risk (LR), defect size A and B, or high-risk (HR), defect size C and D. Inpatient data, including the presence of pulmonary hypertension, extracorporeal life support (ECLS) utilization, and mechanical ventilation days, were collected. Post-discharge data including the prevalence of asthma, pulmonary hypertension, emergency department visits, the total number of hospitalizations, and average rehospitalization days were collected. Frequentist analysis was used.

Results: The outcomes for 133 NICU patients were analyzed (HR: n=54, LR: n=79). During NICU stay, the prevalence of pulmonary hypertension [HR: 16/54 (30%) vs. LR: 9/79 (12%), P=0.009], ECLS utilization [HR: 19/54 (35%) vs. LR: 4/79 (5%), P<0.001], and the average number of mechanical ventilation days [HR: 17 days (IQR: 12-27) vs. LR: 5 days (IQR: 2-9), P<0.001] were significantly higher in the HR CDH group. Post NICU discharge, the prevalence of asthma [HR: 20/54 (37%), vs. LR: 17/79 (22%), P=0.050)] and the total days of rehospitalization [HR: 9 (IQR: 2-27) vs. LR: 4 (IQR: 1-8), P=0.035] were significantly higher in HR group. Of the patients seen in the comprehensive multidisciplinary CDH clinic, obstructive lung disease measured by impulse oscillometry was increased in the HR CDH population compared to the reference group [median R5Hz was 12.95 kPa/(L/s) in CDH vs. 9.8 kPa/(L/s) (P=0.010)].

Conclusions: HR CDHSG Stage is associated with worse inpatient and long-term pulmonary outcomes.

Keywords: Congenital Diaphragmatic Hernia Study Group Staging; Congenital diaphragmatic hernia (CDH); asthma; impulse oscillometry; pulmonary outcomes.

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Conflict of interest statement

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://tp.amegroups.com/article/view/10.21037/tp-23-14/coif). The series “The Impact of the Progresses of Knowledge and Technologies in Pediatrics” was commissioned by the editorial office without any funding or sponsorship. CKJ serves as the Vice President for the Texas Society of Sleep Professionals (non for profit). The authors have no other conflicts of interest to declare.

Figures

Figure 1
Figure 1
CDH defect size categorization into low- and high-risk CDH. Low-risk defects are CDHSG defects (A) and (B). High-risk defects are CDHSG defects (C) and (D). Defects are shown as a left CDH would be viewed from the peritoneal cavity. CDH, congenital diaphragmatic hernia; CDHSG, Congenital Diaphragmatic Hernia Study Group.
Figure 2
Figure 2
Perfusion ratio of the ipsilateral lung in low-risk (CDHSG defects A/B) and high-risk (CDHSG defects C/D) patients. CDH, congenital diaphragmatic hernia; CDHSG, Congenital Diaphragmatic Hernia Study Group.
Figure 3
Figure 3
Abnormal impedance (peripheral airway obstruction) by IOS in patients with high-risk CDH. Increase airway resistance at 5Hz in high-risk CDH patients compared with the reference group (predicted values). No difference in low-risk CDH patients. Fres (resonant frequency) increased in high- and low-risk CDH patients compared with the reference group (predicted values). *, P value <0.05. IOS, impulse oscillometry; CDH, congenital diaphragmatic hernia.
See this image and copyright information in PMC

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