BACKGROUND: Infants with congenital diaphragmatic hernia (CDH) experience high morbidity and mortality due to pulmonary arterial hypertension and hypoplasia. Mechanical ventilation is a central component of CDH management. Our objective was to evaluate the impact of a standardized clinical practice guideline (implemented in January 2012) on ventilator management for infants with CDH, and associate management changes with short-term outcomes, specifically extracorporeal membrane oxygenation (ECMO) utilization and survival to discharge.
METHODS: We conducted a retrospective pre-post study of 103 CDH infants admitted from January 2007-July 2021, divided pre- (n = 40) and post-guideline (n = 63). Clinical outcomes, ventilator settings, and blood gas values in the first 7 days of mechanical ventilation were compared between the pre- and post-guideline cohorts.
RESULTS: Post-guideline, ECMO utilization decreased (11% vs 38%, p = 0.001) and survival to discharge improved (92% vs 68%, p = 0.001). More post-guideline patients remained on conventional mechanical ventilation without need for escalation to high-frequency ventilation or ECMO, and had higher pressures and PaCO2 with lower FiO2 and PaO2 (p < 0.05).
CONCLUSIONS: Standardized ventilator management optimizing pressures for adequate lung expansion and minimizing oxygen toxicity improves outcomes for infants with CDH.
METHODS: III.

BACKGROUND: Infants with congenital diaphragmatic hernia (CDH) are at high risk of death, even despite extracorporeal membrane oxygenation (ECMO) support. In January 2012 we implemented a standardized clinical practice guideline (CPG) to manage infants with CDH. We hypothesized that infants with CDH managed with CPG had better clinical outcomes, less ECMO utilization, and increased survival to discharge.
METHODS: We conducted a retrospective pre-post study of infants with CDH admitted between January 2007 and July 2021 (n = 133). Patients were divided into Cohort 1, pre-CPG (January 2007 to December 2011, n = 54), and Cohort 2, post-CPG (January 2012 to July 2021, n = 79).
RESULTS: More patients in Cohort 1 were small for gestational age than in Cohort 2. No other patient demographics were different between cohorts. Cohort 2 had significantly lower ECMO utilization as compared to Cohort 1 (18% vs 50%, p<0.001). Cohort 2 had significantly higher survival to discharge compared to Cohort 1 (85% vs 57%, p<0.001). Survival for ECMO-treated patients in Cohort 2 was significantly higher than in Cohort 1 (71% vs 26%, p = 0.005). In Cohort 1, 70% of the non-survivors were repaired, of which 81% were repaired on ECMO. In Cohort 2, 8% of the non-survivors were repaired, none on ECMO. Only 3% in Cohort 2 were discharged with pulmonary hypertension medication.
CONCLUSIONS: A standardized CPG to manage patients with CDH decreased ECMO utilization and improved clinical outcomes including survival to discharge. Refinement of management strategies, implementation of new interventions, and meticulous care can improve outcomes in patients with CDH.

We conducted a study of five patients with congenital diaphragmatic hernia (CDH) in the department of antenatal diagnosis over a period of two years. Our study highlights the overall benchmarks for this disease and its management as well as the news on prenatal assessment of neonatal prognosis using Lung Over Head Ratio (LHR): ultrasound versus magnetic resonance imaging (MRI) in the third world. The purpose of this study is to evaluate the prognosis of these new-born babies and support parents of a fetus with diaphragmatic hernia.

Congenital diaphragmatic hernia (CDH) is a birth defect of the diaphragm in which abdominal organs herniate through the defect into the thoracic cavity. The main pathophysiology is respiratory distress and persistent pulmonary hypertension because of pulmonary hypoplasia caused by compression of the elevated organs. Recent progress in prenatal diagnosis and postnatal care has led to an increase in the survival rate of patients with CDH. However, some survivors experience mid- and long-term disabilities and complications requiring treatment and follow-up. In recent years, the establishment of clinical practice guidelines has been promoted in various medical fields to offer optimal medical care, with the goal of improvement of the disease\' outcomes, thereby reducing medical costs, etc. Thus, to provide adequate medical care through standardization of treatment and elimination of disparities in clinical management, and to improve the survival rate and mid- and long-term prognosis of patients with CDH, we present here the clinical practice guidelines for postnatal management of CDH. These are based on the principles of evidence-based medicine using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach. The recommendations are based on evidence and were determined after considering the balance among benefits and harm, patient and society preferences, and medical resources available for postnatal CDH treatment.

OBJECTIVE: We sought to identify implementation barriers and opportunities to increase utilization of the Canadian Congenital Diaphragmatic Hernia (CDH) Collaborative\'s clinical practice guideline.
METHODS: A validated readiness assessment was sent via SurveyMonkey™ to CAPSNet site coordinators and local CDH stakeholders. The survey was open from 11/2018 to 02/2019. Data and responses were analyzed using descriptive statistics (REB 2019-4753).
RESULTS: Eighty-six responses were received, of which 65% (n = 56/86) were fully completed. The greatest number of responses came from neonatology (n = 27), pediatric surgery (n = 25), and respiratory therapy (n = 10). Seventy-eight percent (n = 67/86) of respondents were aware of the CDH guideline, and 63% (n = 54/86) used the entire guideline, while 23% (n = 20/86) used only certain sections. Besides recommendations pertaining to fetal intervention and ECLS, interdisciplinary long-term surveillance and prenatal diagnosis were considered most difficult to implement owing to funding limitations. Most respondents (n = 49/56; 87.5%) felt they could implement >75% of the recommendations. Establishing common team goals [i.e., minimize care variations] (n = 33/58;57%), provider buy-in [commitment of all health professionals to the guideline] (n = 28/58;48%), and regular compliance assessment (n = 23/58;40%) would increase uptake.
CONCLUSIONS: There is national awareness of the CDH guideline. Implementation strategies ensuring common team goals, provider buy-in, and regular compliance assessment should increase guideline uptake/utilization. Consolidating funding for interdisciplinary long-term surveillance and prenatal diagnosis is necessary for any site-specific implementation strategy.
METHODS: Level 4 qualitative, survey.

Standardized care may improve outcomes in many diseases including congenital diaphragmatic hernia (CDH). Our study assesses the variability of CDH clinical practice guidelines (CPG) among North American centers.
North American member institutions of the CDH Study Group and the Pediatric Surgical Research Collaborative were solicited to submit their CDH CPG. Elements from each CPG were collected and classified according to therapeutic purpose. Elements were assigned to umbrella topics of prenatal assessment, delivery plus initial resuscitation, ventilatory and cardiovascular management, therapeutic targets, analgesia, and criteria for transitions in care. Descriptive analyses were performed to characterize the scope and variability of CPGs.
Sixty-eight centers provided 40 responses (59%). Of these, 29 (73%) had a CDH CPG, of which 27 were obtained for review. All CPGs had a primary focus of preoperative care. Conventional ventilation was the first-line strategy in all CPGs. Ninety-three percent reported a peak inspiratory pressure limit (mean: 25.2 ± 2 cm H2O). Target oxygenation and ventilatory variables had low coefficients of variation. Two-thirds of CPGs discussed echocardiography, with indications for inhaled nitric oxide, sildenafil, and prostaglandins detailed in 81%, 30%, and 22% of CPGs, respectively. Extracorporeal life support and operative indications were specified in 93% and 59%, respectively, although specific targets for each were highly variable.
This synthesis of North American CDH CPGs identifies areas of both alignment and variability and provides objective data about individual institutional guidelines in CDH care. These data may inform the development of a consensus-based, multi-institutional approach to standardized CDH management in North America.

BACKGROUND: Pediatric surgeons frequently offer prenatal consultation for congenital pulmonary airway malformation (CPAM) and congenital diaphragmatic hernia (CDH); however, there is no evidence-based consensus to guide prenatal decision making and counseling for these conditions. Eliciting feedback from experts is integral to defining best practice regarding prenatal counseling and intervention.
METHODS: A Delphi consensus process was undertaken using a panel of pediatric surgeons identified as experts in fetal therapy to address current limitations. Areas of discrepancy in the literature on CPAM and CDH were identified and used to generate a list of content and intervention questions. Experts were invited to participate in an online Delphi survey. Items that did not reach first-round consensus were broken down into additional questions, and consensus was achieved in the second round.
RESULTS: Fifty-four surgeons (69%) responded to at least one of the two survey rounds. During round one, consensus was reached on 54 of 89 survey questions (61%), and 45 new questions were developed. During round two, consensus was reached on 53 of 60 survey questions (88%).
CONCLUSIONS: We determined expert consensus to establish guidelines regarding perinatal management of CPAM and CDH. Our results can help educate pediatric surgeons participating in perinatal care of these patients.
METHODS: V.

The Canadian Pediatric Surgery Network (CAPSNet) has been collecting population-based data regarding congenital diaphragmatic hernia (CDH) across its 17 perinatal sites since 2005. With >500 infants registered to date, CAPSNet has addressed many critical knowledge gaps pertaining to CDH care. Most importantly, it has identified variability in both CDH practice and outcome across Canada. Using the successful Evidence-based Practice for Improving Quality (EPIQ) method, CAPSNet is undertaking a national, multidisciplinary effort to standardize best practices for CDH, from prenatal diagnosis to hospital discharge, based on the best available evidence. The present article outlines the value of clinical research networks and the process CAPSNet will undertake to produce national consensus guidelines for CDH care.
Le Réseau canadien de chirurgie pédiatrique recueille des données en population sur la hernie diaphragmatique congénitale (HDC) dans 17 sites périnatals depuis 2005. Puisque plus de 500 nourrissons y sont inscrits jusqu’à présent, le Réseau a corrigé de nombreuses lacunes liées aux soins de la HDC. Qui plus est, il a décelé la variabilité dans la pratique et les résultats de la HDC au Canada. Selon la méthode EPIC (un acronyme anglais qui signifie pratique fondée sur des données probantes pour améliorer la qualité), le Réseau déploie des efforts nationaux et multidisciplinaires pour normaliser les pratiques exemplaires en matière de soins de la HDC, du diagnostic prénatal au congé de l’hôpital, d’après les meilleures données probantes. Le présent article souligne la valeur des réseaux de recherche clinique et le processus que le Réseau entreprendra pour produire des lignes directrices consensuelles nationales sur les soins de la HDC.

Pulmonary hypertension is associated with diverse cardiac, pulmonary, and systemic diseases in neonates, infants, and older children and contributes to significant morbidity and mortality. However, current approaches to caring for pediatric patients with pulmonary hypertension have been limited by the lack of consensus guidelines from experts in the field. In a joint effort from the American Heart Association and American Thoracic Society, a panel of experienced clinicians and clinician-scientists was assembled to review the current literature and to make recommendations on the diagnosis, evaluation, and treatment of pediatric pulmonary hypertension. This publication presents the results of extensive literature reviews, discussions, and formal scoring of recommendations for the care of children with pulmonary hypertension.