Objective The Canadian Congenital Diaphragmatic Hernia (CDH) Collaborative sought to make its existing clinical practice guideline, published in 2018, into a ‘living document’. Design and main outcome measures Critical appraisal of CDH literature adhering to Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology. Evidence accumulated between 1 January 2017 and 30 August 2022 was analysed to inform changes to existing or the development of new CDH care recommendations. Strength of consensus was also determined using a modified Delphi process among national experts in the field. Results Of the 3868 articles retrieved in our search that covered the 15 areas of CDH care, 459 underwent full-text review. Ultimately, 103 articles were used to inform 20 changes to existing recommendations, which included aspects related to prenatal diagnosis, echocardiographic evaluation, pulmonary hypertension management, surgical readiness criteria, the type of surgical repair and long-term health surveillance. Fifteen new CDH care recommendations were also created using this evidence, with most related to the management of pain and the provision of analgesia and neuromuscular blockade for patients with CDH. Conclusions The 2023 Canadian CDH Collaborative’s clinical practice guideline update provides a management framework for infants and children with CDH based on the best available evidence and expert consensus.

Pulmonary vascular disease and resultant pulmonary hypertension (PH) have been increasingly recognized in the preterm population, particularly among patients with bronchopulmonary dysplasia (BPD). Limited data exist on the impact of PH severity and right ventricular (RV) dysfunction at PH diagnosis on outcome. The purpose of this study was to evaluate if echocardiography measures of cardiac dysfunction and PH severity in BPD‐PH were associated with mortality. The study is a retrospective analysis of the echocardiography at three months or less from time of PH diagnosis. Survival analysis using a univariate Cox proportional hazard model is presented and expressed using hazard ratios (HR). We included 52 patients with BPD and PH of which 16 (31%) died at follow‐up. Average gestational age at birth was 26.3 ± 2.3 weeks. Echocardiography was performed at a median of 43.3 weeks (IQR: 39.0–54.7). The median time between PH diagnosis and death was 117 days (range: 49–262 days). Multiple measures of PH severity and RV performance were associated with mortality (sPAP/sBP: HR 1.02, eccentricity index: HR 2.02, tricuspid annular plane systolic excursion Z‐score: HR 0.65, fractional area change: HR 0.88, peak longitudinal strain: HR 1.22). Hence, PH severity and underlying RV dysfunction at PH diagnosis were associated with mortality in BPD‐PH patients. While absolute estimation of pulmonary pressures is not feasible in every screening echocardiography, thorough evaluation of RV function and other markers of PH may allow to discriminate the most at‐risk population and should be considered as standard add‐ons to the current screening at 36 weeks.