Fetal biophysical predictors of pulmonary hypertension severity, management, and treatment in infants born with congenital diaphragmatic hernia.

Background: Pulmonary hypertension (PHTN) causes significant morbidity and mortality in patients with congenital diaphragmatic hernia (CDH). Currently, there is no routinely obtained prenatal prognostic marker to reliably predict postnatal CDH-associated PHTN severity.

Methods: The CDH Study Group (CDHSG) registry was queried for infants born from 2015 to 2021 with a graded (1-4) PHTN diagnosis.

Congenital diaphragmatic hernia-associated pulmonary hypertension.

Congenital diaphragmatic hernia (CDH) is characterized by a developmental insult which compromises cardiopulmonary embryology and results in a diaphragmatic defect, allowing abdominal organs to herniate into the hemithorax. Among the significant pathophysiologic components of this condition is pulmonary hypertension (PH), alongside pulmonary hypoplasia and cardiac dysfunction. Fetal pulmonary vascular development coincides with lung development, with the pulmonary vasculature evolving alongside lung maturation.

The CDH Study Group: Past, Present, and Future.

The Congenital Diaphragmatic Hernia Study Group (CDHSG) is an international consortium of medical centers actively collecting and voluntarily contributing data pertaining to live born congenital diaphragmatic hernia (CDH) patients born and/or managed at their institutions. These data are aggregated to construct a comprehensive registry that participating centers can access to address specific clinical inquiries and track patient outcomes. Since its establishment in 1995, 147 centers have taken part in this initiative, including 53 centers from 17 countries outside the United States, with 95 current active centers across the globe.

Extracorporeal life support in congenital diaphragmatic hernia.

Congenital diaphragmatic hernia (CDH) is the most common indication for ECLS in neonatal respiratory failure. The ultimate purpose of ECLS is to grant cardiopulmonary support, allowing time for operative intervention and optimization of cardiopulmonary function as the pathophysiologic processes of pulmonary hypertension, pulmonary hypoplasia, and ventricular dysfunction either improve or resolve. In CDH, ECLS plays a crucial role in the management of the most challenging patients, facilitating postnatal stabilization, allowing a ventilation strategy which minimizes barotrauma and volutrauma, and permitting treatment of and recovery from pulmonary hypertension and/or cardiac dysfunction.

Recent advances in the treatment of complex congenital diaphragmatic hernia-a narrative review.

Background And Objective: Congenital diaphragmatic hernia (CDH) is an anomaly of the cardiopulmonary system maturation process that results from both a global embryopathy and concomitant mechanical compression of the cardiopulmonary system from the abdominal contents during fetal maturation. This results in pulmonary hypertension, pulmonary hypoplasia, and cardiac dysfunction, requiring intense critical care management. The patients with highest risk CDH are the most challenging, resource-intensive, and bear most of the mortality.

Ethical Considerations in Critically Ill Neonatal and Pediatric Patients.

The care of critically ill neonates and pediatric patients can be particularly emotionally and ethically challenging. Emerging evidence suggests that we can improve the patient, family, and care team experience in the critical care setting through a better understanding and application of ethical frameworks and communication strategies. We conducted a multidisciplinary panel session at the American Academy of Pediatrics National Conference and Exhibition in the fall of 2022 wherein we explored a myriad of ethical and communication considerations in this unique patient population, with congenital diaphragmatic hernia (CDH) as the congenital anomaly/disease framework.

Isolated aortic arch anomalies are associated with defect severity and outcome in patients with congenital diaphragmatic hernia.

Purpose: Congenital diaphragmatic hernia (CDH) patients often have suspected isolated aortic arch anomalies (IAAA) on imaging. The purpose of this work was to describe the incidence and outcomes of CDH + IAAA patients.

Methods: Cardiovascular data were collected for infants from the CDH Study Group born between 2007 and 2019.

Association of timing of congenital diaphragmatic hernia repair with survival and morbidity for patients not requiring extra-corporeal life support.

Background: While physiologic stabilization followed by repair has become the accepted paradigm for management of congenital diaphragmatic hernia (CDH), few studies have examined the effect of incremental changes in operative timing on patient outcomes. We hypothesized that later repair would be associated with higher morbidity and mortality.

Methods: Data were queried from the CDH Study Group (CDHSG) from 2007-2020.

Impact of Repeat Extracorporeal Life Support on Mortality and Short-term In-hospital Morbidities in Neonates With Congenital Diaphragmatic Hernia.

Objective: To evaluate the impact of repeat extracorporeal life support (ECLS) on survival and in-hospital outcomes in the congenital diaphragmatic hernia (CDH) neonates.

Background: Despite the widespread use of ECLS, investigations on multiple ECLS courses for CDH neonates are limited.

Methods: This is a retrospective cohort study of all ECLS-eligible CDH neonates enrolled in the Congenital Diaphragmatic Hernia Study Group registry between 1995 and 2019.

Neonatal rodent ventilation and clinical correlation in congenital diaphragmatic hernia.

Introduction: Ventilator management is a critical part of managing congenital diaphragmatic hernia (CDH). We aimed to use a murine model and patient data to study CDH-associated differences in oxygenation, airway resistance, and pulmonary mechanics by disease severity.

Methods: We used the nitrofen model of CDH.

Rapunzel Syndrome Complicated by Sepsis, Bowel Perforation, and Pancreatitis in an 11-year-old Malnourished Female.

The most common presenting symptoms of Rapunzel syndrome include abdominal pain (37%), nausea and vomiting (33.3%), obstruction (25.9%), and peritonitis (18.

Health Equity and Social Determinants of Health in Pediatric Gastroenterology.

Social determinants of health (SDH) as outlined by Healthy People 2020 encompasses 5 key domains: economic, education, social and community context, health and health care, and neighborhood and built environment. This article emphasizes pediatric populations and some of the existing SDH and health care disparities seen in pediatric gastroenterology. We specifically review inflammatory bowel disease, endoscopy, bariatric surgery, and liver transplantation.

Randomized controlled trial of the "WISER" intervention to reduce healthcare worker burnout.

Objective: Test web-based implementation for the science of enhancing resilience (WISER) intervention efficacy in reducing healthcare worker (HCW) burnout.

Design: RCT using two cohorts of HCWs of four NICUs each, to improve HCW well-being (primary outcome: burnout). Cohort 1 received WISER while Cohort 2 acted as a waitlist control.

Telemedicine as a component of forward triage in a pandemic.

Objective(s): Coronavirus disease 2019 (COVID-19) presents an enormous challenge to healthcare systems globally. Optimizing access to healthcare while minimizing face-to-face patient encounters is critical to limiting exposures, conserving resources, and preserving health. We aimed to evaluate the utility of a COVID-focused telehealth program in avoiding potential in-person visits while maintaining high patient satisfaction.

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.

Incidence and outcomes of patients with congenital diaphragmatic hernia and pulmonary sequestration.

Background: Bronchopulmonary sequestration (BPS) has long been identified in patients with congenital diaphragmatic hernia (CDH), however the reported incidence in the literature varies widely and is not based on large series.

Methods: Version 4 of the Congenital Diaphragmatic Hernia Study Group (CDHSG) included questions specifically identifying BPS-associated cases. The data were prospectively collected between 2015 and 2020.

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.

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.

Extracellular vesicles influence the pulmonary arterial extracellular matrix in congenital diaphragmatic hernia.

Objective: Abnormal pulmonary vasculature directly affects the development and progression of congenital diaphragmatic hernia (CDH)-associated pulmonary hypertension (PH). Though overarching structural and cellular changes in CDH-affected pulmonary arteries have been documented, the precise role of the extracellular matrix (ECM) in the pulmonary artery (PA) pathophysiology remains undefined. Here, we quantify the structural, compositional, and mechanical CDH-induced changes in the main and distal PA ECM and investigate the efficacy of mesenchymal stem cell-derived extracellular vesicles (MSC-EVs) as a therapy to ameliorate pathological vascular ECM changes.