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Pulmonary outcomes of congenital diaphragmatic hernia patients based on defect size (CDH Study Group Stage)
Hina Emanuel1, Hannah V Breitschopf2, Matthew T Harting2
1Division of Pulmonary, Allergy & Immunology, and Sleep Medicine, Department of Pediatrics, McGovern Medical School at the University of Texas Health Science Center at Houston (UTHealth Houston) and Children's Memorial Hermann Hospital, Houston, TX, USA.
Insights
High-risk congenital diaphragmatic hernia (CDH) patients experience worse inpatient and long-term pulmonary outcomes, including higher rates of pulmonary hypertension and asthma. Defect size significantly impacts congenital diaphragmatic hernia (CDH) patient morbidity.
Area of Science:
- Pediatric Surgery
- Neonatology
- Pulmonology
Background:
- Congenital diaphragmatic hernia (CDH) is linked to significant pulmonary complications.
- Previous research indicates a correlation between inpatient morbidity and the size of the diaphragmatic defect.
- This study aimed to assess long-term pulmonary outcomes based on CDH Study Group (CDHSG) defect size.
Purpose of the Study:
- To evaluate the association between Congenital Diaphragmatic Hernia Study Group (CDHSG) defect size and long-term pulmonary outcomes.
- To compare inpatient and post-discharge pulmonary morbidity in low-risk (LR) versus high-risk (HR) CDH patients.
Main Methods:
- Retrospective analysis of 133 CDH patients managed in a NICU and followed in a multidisciplinary CDH clinic.
- Patients stratified into LR (defect size A/B) and HR (defect size C/D) groups based on CDHSG criteria.
- Collected inpatient data (pulmonary hypertension, ECLS, ventilation days) and post-discharge data (asthma, rehospitalizations, obstructive lung disease).
Main Results:
- High-risk (HR) CDH patients showed significantly higher rates of pulmonary hypertension, ECLS utilization, and mechanical ventilation days during NICU stay.
- Post-discharge, HR CDH patients had a higher prevalence of asthma and increased total rehospitalization days.
- Obstructive lung disease was more prevalent in the HR CDH group compared to controls.
Conclusions:
- High-risk CDHSG Stage is associated with worse inpatient pulmonary morbidity.
- Congenital diaphragmatic hernia (CDH) defect size is a significant predictor of long-term pulmonary outcomes.
- Early identification and management strategies for HR CDH patients are crucial for improving long-term pulmonary health.
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.
