Congenital Diaphragmatic Hernia Guidelines Compliance and Outcomes in Canada.

BACKGROUND

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.

METHOD: 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).

RESULTS

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.

CONCLUSIONS

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.