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. IAAA were defined as coarctation of aorta, hypoplastic aortic arch, interrupted aortic arch, and aortic aneurysmal disease on early, postnatal echocardiography. Patients with major cardiac malformations and/or chromosomal abnormalities were excluded. Primary outcomes included the rate of aortic intervention, rates of extracorporeal life support (ECLS) utilization, and mortality. RESULTS: Of 6357 CDH infants, 432 (7%) were diagnosed with a thoracic aortic anomaly. Of these, 165 were diagnosed with IAAA, most commonly coarctation of the aorta (n = 106; 64%) or hypoplastic aortic arch (n = 58; 35%). CDH + IAAA patients had lower birthweights (3 kg vs. 2.9 kg) and Apgar scores (7 vs. 6) than patients without IAAA (both χ2 p < 0.001). CDH + IAAA were less likely to undergo diaphragm repair (72 vs. 87%, p < 0.001), and overall mortality was higher for CDH + IAAA infants (58 vs. 24%, p < 0.001). When controlling for defect size, birth weight, and Apgar, IAAA were significantly associated with mortality (OR 3.3, 95% CI 2.2-5.0; p < 0.01) but not associated with ECLS (OR 0.98, 95% CI 0.65-1.50; p = 0.90). Only 17% (n = 28) of CDH + IAAA patients underwent aortic intervention. CONCLUSIONS: IAAA in CDH are associated with increased mortality. This often simply reflects severity of the defect and thoracic anatomic derangement, as opposed to unique aortic pathology, given few CDH + IAAA patients undergo aortic intervention.

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. For control and CDH rodents, data were collected within the first hour of life. Oxygen saturations (SpO2 ) were collected using MouseOx, and large airway resistance and inspiratory capacities were collected using flexiVent. A single-center, retrospective review of term CDH infants from 2014 to 2020 was performed. Tidal volumes were collected every 6 h for the first 48 h of life or until the patient was taken off conventional ventilation. Newborns that were mechanically ventilated but had no pulmonary pathology were used as controls. CDH severity was defined using the CDH Study Group (CDHSG) classification system. RESULTS: Control rodents had a median SpO2 of 94% (IQR: 88%-98%); CDH pups had a median SpO2 of 27.9% (IQR: 22%-30%) (p < 0.01). CDH rodents had lower inspiratory capacity than controls (median: 110 µl, IQR: 70-170 vs. median: 267 µl, IQR: 216-352; p < 0.01). CDH infants had a lower initial SpO2 than control infants. Overall, CDH infants had lower tidal volumes than control infants (median: 4.2 ml/kg, IQR: 3.3-5.0 vs. 5.4 ml/kg, IQR: 4.7-6.2; p = 0.03). Tidal volumes varied by CDHSG stage. CONCLUSION: Newborns with CDH have lower SpO2 and lower, CDHSG stage specific, tidal volumes than control infants. The nitrofen model of CDH reflects these differences. Rodent models may be useful in studying therapeutic ventilatory strategies for CDH infants.

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. METHODS: All patients with COVID-related virtual visits at our center between March and May 2020 were included. Demographic, satisfaction, and clinical information were gathered using a modified, validated telehealth satisfaction questionnaire disseminated via email or telephone. Data were analyzed using Stata. RESULTS: Of 581 eligible patients, 180 (31%) responded to the survey. Symptoms (73%) and possible exposure (22%) were the main reasons cited for pursuing a virtual visit; cough (44%) and fever (36%) were the most common presenting symptoms. Regarding patient satisfaction, most patients rated the experience as "very good" or "excellent", and 94% of respondents said they would recommend COVID-focused triage through telehealth to others. Over 81% of patients indicated that, if telehealth was not an option, they would have sought an in-person encounter. Ultimately, only 27% of patients reported pursuing a face-to-face encounter after participating in the virtual visit. CONCLUSION: Based on patient self-reporting, telemedicine potentially prevented face-to-face COVID-related encounters. Patients expressed satisfaction with the virtual process and were less likely to pursue in-person consultation. Leveraging a telehealth strategy for forward triage has the potential to reduce exposures while conserving healthcare resources.