Congenital Diaphragmatic Hernia Guidelines Compliance and Outcomes in Canada

Perinatal congenital diaphragmatic hernia (CDH) care in Canada has been supported by comprehensive management guidelines published in 2018, with variable implementation/uptake across sites. We evaluated institutional compliance across 8 Key Performance Indicators (KPIs) and assessed differences in clinical outcomes.

ology: The prospective CAPSNet patient database was interrogated. Patients pre- (2013-17) and post-guideline (2019-2023) publication were compared with a 1-year intervening washout. Neonates admitted to sites reporting >10 cases over each study epoch were included (8/16). The 8 KPI's addressed (1) prenatal diagnosis and risk stratification; (2) prenatal echocardiography; (3) prenatal genetic testing; (4) preoperative echocardiography; (5) use of GORE-tex™ or muscle flaps for non-primary repair; (6) pre-discharge echocardiography; (7) diagnosis and management of pulmonary hypertension; and (8) structured, long-term interdisciplinary follow-up. KPI compliance was dichotomized and defined as >70% of neonates exposed to the specific KPI. An institution was deemed "guidelines compliant" if >6/8 KPIs were achieved. Standard statistical analyses were performed, including regression analyses to calculate adjusted odds ratios (aOR).

The study cohort included 213 and 166 patients pre- and post-guideline publication, respectively. Overall network mortality was similar pre- (36/213;16.9%) and post-guideline (22/166;13.3%) publication. There was inter-site variability in individual KPI responsiveness to guidelines publication in both guidelines compliant (n=2) and non-compliant (n=6) sites. However, the two sites that achieved overall guidelines compliance (n=114 comprising both pre- and post-guideline epochs) demonstrated a 3-fold pre- to post-implementation decrease in overall mortality (11.5% v 3.8%, aOR 0.36, [CI 0.06, 2.06]). Furthermore, compared to sites that remained non-compliant after guidelines publication, compliant sites demonstrated a lower mortality rate (3.8% v 17.7% aOR 5.51 [CI 1.2 - 25.38[) after adjustment using the CDH Study Group Mortality Prediction Score. Secondary outcomes (length of stay, aggregate complications, oxygen supplementation, enteral access) between compliant and non-compliant sites did not demonstrate a difference.

Adherence to guidelines-driven KPIs is associated with improved survival in Canadian CDH patients. Dissemination and implementation of existing guidelines recommendations, and understanding both barriers and enablers of site-specific adoption can meaningfully impact patient outcomes.