Related Experiment Video
Updated: Oct 26, 2025
Transuterine Fetal Tracheal Occlusion Model in Mice
Published on: February 5, 2021
Mortality in Congenital Diaphragmatic Hernia: A Multicenter Registry Study of Over 5000 Patients Over 25 Years
Vikas S Gupta1, Matthew T Harting1, Pamela A Lally1
1Department of Pediatric Surgery, McGovern Medical School at the, University of Texas Health Science Center and Children's Memorial Hermann, Hospital, Houston, Texas.
Insights
Risk-adjusted survival for congenital diaphragmatic hernia (CDH) patients has improved over 25 years. This study shows better observed-to-expected mortality rates in long-term CDH Study Group participants.
Area of Science:
- Pediatric Surgery
- Neonatal Care
- Congenital Anomalies
Background:
- Congenital diaphragmatic hernia (CDH) is a serious condition affecting newborns.
- Despite advances, CDH mortality rates have appeared to plateau in recent years.
- Long-term, consistent participation in multicenter collaborations like the CDH Study Group (CDHSG) is crucial for tracking outcomes.
Purpose of the Study:
- To evaluate if risk-adjusted survival for CDH patients has improved over the past 25 years.
- To analyze mortality trends within centers consistently participating in the CDHSG.
- To assess changes in CDH characteristics and management strategies over time.
Main Methods:
- Analysis of CDHSG registry data from 1995 onwards, divided into 5-year intervals.
- Risk stratification of patients using a validated score based on 5-minute Apgar (Apgar5) and birth weight.
- Creation of a risk-adjusted, observed-to-expected (O:E) mortality model with the earliest era as reference.
Main Results:
- 5203 patients from 23 centers with over 22 years of participation were analyzed.
- Overall CDH mortality decreased from 30.7% in Era 1 to 25.8% in Era 5 (P=0.03).
- Risk-adjusted mortality significantly improved in Era 5 compared to Era 1 (OR 0.78, P=0.03), with O:E mortality also showing improvement.
Conclusions:
- Risk-adjusted and observed-to-expected mortality for congenital diaphragmatic hernia have improved over the study period.
- These findings highlight the benefits of long-term participation in collaborative research for improving infant outcomes.
- Changes in surgical strategies, including increased minimally invasive repairs and later repair timing, may have contributed to improved survival.
Objective:
To determine if risk-adjusted survival of patients with CDH has improved over the last 25 years within centers that are long-term, consistent participants in the CDH Study Group (CDHSG).
Summary Background Data:
The CDHSG is a multicenter collaboration focused on evaluation of infants with CDH. Despite advances in pediatric surgical and intensive care, CDH mortality has appeared to plateau. Herein, we studied CDH mortality rates amongst long-term contributors to the CDHSG.
Methods:
We divided registry data into 5-year intervals, with Era 1 (E1) beginning in 1995, and analyzed multiple variables (operative strategy, defect size, and mortality) to assess evolution of disease characteristics and severity over time. For mortality analyses, patients were risk stratified using a validated prediction score based on 5-minute Apgar (Apgar5) and birth weight. A risk-adjusted, observed to expected (O:E) mortality model was created using E1 as a reference.
Results:
5203 patients from 23 centers with >22years of participation were included. Birth weight, Apgar5, diaphragmatic agenesis, and repair rate were unchanged over time (all P > 0.05). In E5 compared to E1, minimally invasive and patch repair were more prevalent, and timing of diaphragmatic repair was later (all P < 0.01). Overall mortality decreased over time: E1 (30.7%), E2 (30.3%), E3 (28.7%), E4 (26.0%), E5 (25.8%) ( P = 0.03). Risk-adjusted mortality showed a significant improvement in E5 compared to E1 (OR 0.78, 95% CI 0.62-0.98; P = 0.03). O:E mortality improved over time, with the greatest improvement in E5.
Conclusions:
Risk-adjusted and observed-to-expected CDH mortality have improved over time.
