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

Ann Surg

. 2021 Jul 29. doi: 10.1097/SLA.0000000000005113. Online ahead of print.

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

Vikas S Gupta 1Matthew T HartingPamela A LallyCharles C MillerRonald B HirschlCarl F DavisMelvin S Dassinger 3rdTerry L BuchmillerKrisa P Van MeursBradley A YoderMichael J StewartKevin P LallyCongenital Diaphragmatic Hernia Study GroupAffiliations expand


Objective: To determine if risk-adjusted survival of patients with congenital diaphragmatic hernia (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 five-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: 5,203 patients from 23 centers with ≥22 years 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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