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Multicenter Study
. 2023 Mar 1;277(3):520-527.
doi: 10.1097/SLA.0000000000005113. Epub 2021 Jul 29.

Mortality in Congenital Diaphragmatic Hernia: A Multicenter Registry Study of Over 5000 Patients Over 25 Years

Vikas S Gupta  1 , Matthew T Harting  1 , Pamela A Lally  1 , Charles C Miller  2 , Ronald B Hirschl  3 , Carl F Davis  4 , MelvinIII S Dassinger  5 , Terry L Buchmiller  6 , Krisa P Van Meurs  7 , Bradley A Yoder  8 , Michael J Stewart  9 , Kevin P Lally  1 ; Congenital Diaphragmatic Hernia Study Group
Affiliations
Multicenter Study

Mortality in Congenital Diaphragmatic Hernia: A Multicenter Registry Study of Over 5000 Patients Over 25 Years

Vikas S Gupta et al. Ann Surg. .

Abstract

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.

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Conflict of interest statement

The authors have no conflicts of interest to disclose.

References

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    1. Lally PA, Skarsgard ED. Congenital diaphragmatic hernia: the role of multi-institutional collaboration and patient registries in supporting best practice. Semin Pediatr Surg. 2017;26:129–135.
    1. Hagadorn JI, Brownell EA, Herbst KW, et al. Trends in treatment and inhospital mortality for neonates with congenital diaphragmatic hernia. J Perinatol. 2015;35:748–754.
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    1. Harting MT, Lally KP. The congenital diaphragmatic hernia study group registry update. Semin Fetal Neonatal Med. 2014;19:370–375.

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