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

Transl Pediatr

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

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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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