Image-based prenatal predictors of postnatal survival, extracorporeal life support, and defect size in right congenital diaphragmatic hernia.

Objective: To determine the association between prenatal ultrasound (US) and magnetic resonance imaging (MRI) characteristics in right congenital diaphragmatic hernia (RCDH) with postnatal outcome.

Study Design: CDH Study Group data were reviewed for all RCDH infants (n = 156) born between 2015 and 2019. Prenatal US and MRI lung size measurements were correlated with survival, extracorporeal life support (ECLS), and defect size.

Image-based prenatal predictors correlate with postnatal survival, extracorporeal life support use, and defect size in left congenital diaphragmatic hernia.

Objective: To evaluate the association between prenatal imaging predictors of patients with left-sided congenital diaphragmatic hernia (LCDH) and postnatal outcomes.

Study Design: CDH study group data were reviewed for LCDH infants born 2015-2019. Prenatal ultrasound (US) and magnetic resonance imaging (MRI) data were collected and correlated with postnatal information including CDHSG defect size (A through D or non-repair (NR)).

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

Objective: To determine if risk-adjusted survival of patients with 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.

Birth weight predicts patient outcomes in infants who undergo congenital diaphragmatic hernia repair.

Purpose: The purpose of this study was to analyze the clinical characteristics and outcomes of low birthweight (LBW) infants with congenital diaphragmatic hernia (CDH) compared to normal birthweight (NBW) infants with CDH. We hypothesized that LBW was associated with increased mortality, decreased extracorporeal life support (ECLS) utilization, and increased pulmonary morbidity in CDH patients.

Methods: Patients in the CDH Study Group from 2007 to 2018 were included.

Early, Postnatal Pulmonary Hypertension Severity Predicts Inpatient Outcomes in Congenital Diaphragmatic Hernia.

Introduction: Pulmonary hypertension (PH) is the major pathophysiologic consequence of congenital diaphragmatic hernia (CDH). We aimed to evaluate the association between early CDH-associated PH (CDH-PH) and inpatient outcomes.

Methods: The CDH Study Group registry was queried for infants born 2015-2019 with echocardiograms before 48h of life.

Elevated proBNP levels are associated with disease severity, cardiac dysfunction, and mortality in congenital diaphragmatic hernia.

Background: Cardiac dysfunction is a key determinant of outcome in congenital diaphragmatic hernia (CDH). Pro-b-type natriuretic peptide (proBNP) is used as a prognosticator in heart failure and cardiomyopathy. We hypothesized that proBNP levels would be associated with ventricular dysfunction and high-risk disease in CDH.

Survival Benefit Associated With the Use of Extracorporeal Life Support for Neonates With Congenital Diaphragmatic Hernia.

Objective: To measure the survival among comparable neonates with CDH supported with and without ECLS.

Summary Of Background Data: Despite widespread use in the management of newborns with CDH, ECLS has not been consistently associated with improved survival.

Methods: A retrospective cohort study was performed using ECLS-eligible CDH Study Group registry patients born between 2007 and 2019.

Predicting lethal pulmonary hypoplasia in congenital diaphragmatic hernia (CDH): Institutional experience combined with CDH registry outcomes.

Background: The Severe Pulmonary Hypoplasia and Evaluation for Resuscitative Efforts (SPHERE) protocol was developed to attempt to identify CDH patients with likely lethal pulmonary hypoplasia. We present our experience with this protocol and utilize the CDH Registry to critically assess the protocol.

Methods: SPHERE patients identified based on prenatal imaging (10/2009-1/2018) were offered ECMO if meeting postnatal physiologic criteria, while others received comfort measures.

Cornelia de Lange syndrome and congenital diaphragmatic hernia.

Purpose: There is a known association between Cornelia de Lange syndrome (CdLS) and congenital diaphragmatic hernia (CDH), with CDH being the cause of death in 5%-20% of CdLS cases. We aimed to identify and describe patients with CDLS and CDH. We hypothesized that CdLS would be associated with high-risk CDH and poor outcomes.

Early Left Ventricular Dysfunction and Severe Pulmonary Hypertension Predict Adverse Outcomes in "Low-Risk" Congenital Diaphragmatic Hernia.

Objectives: Given significant focus on improving survival for "high-risk" congenital diaphragmatic hernia, there is the potential to overlook the need to identify risk factors for suboptimal outcomes in "low-risk" congenital diaphragmatic hernia cases. We hypothesized that early cardiac dysfunction or severe pulmonary hypertension were predictors of adverse outcomes in this "low-risk" congenital diaphragmatic hernia population.

Design: This is a retrospective cohort study using data from the Congenital Diaphragmatic Hernia Study Group registry.

Congenital diaphragmatic hernia and associated omphalocele: a study from the CDHSG registry.

Background: Congenital Diaphragmatic Hernia (CDH) associated with Omphalocele is a rare condition, and only a few case reports are available in the literature. Both conditions are associated with some degree of pulmonary hypoplasia. We hypothesize that the combination of CDH with Omphalocele might be associated with poorer outcomes.

Clinical features and outcomes associated with tracheostomy in congenital diaphragmatic hernia.

Introduction: The purpose of this study was to examine the clinical features/outcomes associated with tracheostomy in infants with congenital diaphragmatic hernia (CDH).

Methods: The study population consisted of liveborn infants reported to the CDH Study Group registry between 2007 and 2017. Subjects were identified as having a tracheostomy if they were discharged or transferred to another hospital with tracheostomy and/or on mechanical ventilation.

Surgical Repair of Congenital Diaphragmatic Hernia After Extracorporeal Membrane Oxygenation Cannulation: Early Repair Improves Survival.

Objective: To determine the optimal timing of congenital diaphragmatic hernia (CDH) repair after extracorporeal membrane oxygenation (ECMO) cannulation.

Summary Background Data: The timing of CDH repair after ECMO cannulation remains a controversial topic due to studies with low power or strong selection bias.

Methods: This is a 2-aim retrospective cohort study based on the CDH Study Group registry for the period of 2007-2017.

Ventricular Dysfunction Is a Critical Determinant of Mortality in Congenital Diaphragmatic Hernia.

Congenital diaphragmatic hernia (CDH) is an anomaly with a high morbidity and mortality. Cardiac dysfunction may be an important and underrecognized contributor to CDH pathophysiology and determinant of disease severity. Our aim was to investigate the association between early, postnatal ventricular dysfunction and outcome among infants with CDH.

Toward Standardized Management of Congenital Diaphragmatic Hernia: An Analysis of Practice Guidelines.

Background: 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.

Methods: North American member institutions of the CDH Study Group and the Pediatric Surgical Research Collaborative were solicited to submit their CDH CPG.

Early CDH repair on ECMO: Improved survival but no decrease in ECMO duration (A CDH Study Group Investigation).

Purpose: "Early on-ECMO" repair of CDH entails repair within 48-72 h of cannulation in an effort to optimize pulmonary physiology, shorten ECMO duration, and, ultimately, improve survival. This study evaluated the effect of early on-ECMO repair as compared to leaving patients unrepaired during ECMO.

Methods: The CDH Study Group database was queried for CDH patients requiring ECMO who either underwent repair within the first 72 h after cannulation or remained unrepaired on ECMO.

Risk-stratification enables accurate single-center outcomes assessment in congenital diaphragmatic hernia (CDH).

Background: Management of CDH is highly variable from center to center, as are patient outcomes. The purpose of this study was to examine risk-stratified survival and extracorporeal membrane oxygenation (ECMO) rates at a single center, and to determine whether adverse outcomes are related to patient characteristics or management.

Methods: A retrospective single-center review of CDH patients was performed, and outcomes compared to those reported by the CDH Study Group (CDHSG) registry.

Lowest PaCO2 on the first day of life predicts mortality and morbidity among infants with congenital diaphragmatic hernia.

Objective: To evaluate the association between hypercarbia in the first 24 h of life and clinical outcomes in infants with congenital diaphragmatic hernia (CDH).

Study Design: Retrospective review of patients entered into the CDHSG registry between 2007-2014. Half of the identified patients were analyzed to identify the PaCO2 value most predictive of mortality.

Evaluating the utility of the "late ECMO repair": a congenital diaphragmatic hernia study group investigation.

Purpose: Optimal timing of congenital diaphragmatic hernia (CDH) repair in patients requiring extracorporeal membrane oxygenation (ECMO) remains controversial. The "late ECMO repair" is an approach where the patient, once deemed stable for decannulation, is repaired while still on ECMO to enable expeditious return to ECMO if surgery induces instability. The goal of this study was to investigate the potential benefit of this approach by evaluating the rate of return to ECMO after repair.

Extracorporeal Membrane Oxygenation (ECMO) Risk Stratification in Newborns with Congenital Diaphragmatic Hernia (CDH).

Background: A means for early postnatal stratification of ECMO risk in CDH newborns could be used to comparatively assess the utilization and outcomes of ECMO use between centers. While multiple CDH mortality risk calculators are available, no validated tool exists specifically for prediction of ECMO use. The purpose of this study was to derive and validate an ECMO risk stratification model.

Prenatally versus postnatally diagnosed congenital diaphragmatic hernia - Side, stage, and outcome.

Aim: To compare outcomes between prenatally and postnatally diagnosed CDH in a large multicenter database of prospectively collected data and evaluate factors associated with poorer outcome for prenatally diagnosed CDH.

Material And Methods: We used information from the multicenter, multinational CDH Study Group database on patients born between 2007 and 2015. We compared differences between prenatally and postnatally diagnosed CDH with respect to survival, side, size, ECMO needs, associated major cardiac malformations and liver position.

Right versus left congenital diaphragmatic hernia - What's the difference?

Background: Right-sided congenital diaphragmatic hernias (CDH) and bigger defect sizes have been associated with poorer outcomes.

Aim: The aim of this study was to evaluate right- and left-sided CDH in terms of size, survival, associated anomalies, and morbidity.

Material And Methods: We used information from a multicenter, multinational database including patients with CDH born between 2007 and 2015.

Treatment Strategies for Congenital Diaphragmatic Hernia: Change Sometimes Comes Bearing Gifts.

Objective: To report treatment strategies' evolution and its impact on congenital diaphragmatic hernia (CDH) outcome.

Design: Registry-based cohort study using the CDH Study Group database, 1995-2013.

Setting: International multicenter database.

Congenital diaphragmatic hernia: The role of multi-institutional collaboration and patient registries in supporting best practice.

Among congenital malformations, congenital diaphragmatic hernia (CDH) is distinguished by its relatively low occurrence rate, need for resource intensive, integrated multidisciplinary care, and widespread variation in practice and outcome. Although randomized controlled trials (RCTs) are considered the gold standard for generating evidence, they are poorly suited to the study of a condition like CDH due to challenges in illness severity adjustment, unpredictability in clinical course and the impact limitations of studying a single intervention at a time. An alternative to RCTs for comparative effectiveness research for CDH is the patient registry, which aggregates multi-institutional condition-specific patient level data into a large CDH-specific database for the dual purposes of collaborative research and quality improvement across participating sites.

Factors associated with early recurrence after congenital diaphragmatic hernia repair.

Background: The purpose of this study was to identify patient and treatment characteristics associated with early (in hospital) hernia recurrence after congenital diaphragmatic hernia (CDH) repair.

Methods: Data from the Congenital Diaphragmatic Hernia Study Group registry were queried from 2007 to 2015. Recurrence of the diaphragmatic hernia after initial repair and prior to death or discharge was determined at the time of reoperation.