In persistent pulmonary hypertension of the newborn (PPHN), the ratio of pulmonary vascular resistance to systemic vascular resistance is increased. Extrapulmonary shunts (patent ductus arteriosus and patent foramen value) allow for right-to-left shunting and hypoxaemia. Systemic hypotension can occur in newborns with PPHN due to variety of reasons, such as enhanced peripheral vasodilation, impaired left ventricular function and decreased preload. Systemic hypotension can lead to end organ injury from poor perfusion and hypoxaemia in the newborn with PPHN. Thus, it must be managed swiftly. However, not all newborns with PPHN and systemic hypotension can be managed the same way. Individualised approach based on physiology and echocardiographic findings are necessary to improve perfusion to essential organs. Here we present a review of the physiology and mechanisms of systemic hypotension in PPHN, which can then guide treatment.

Reduced heart rate variability (HRV), anindicator of autonomic nervous systemdysfunction, can be used to identify critically ill patients at higher risk for adverse outcomes.Limited data exists on HRV in children withcardiac disease. Early detection of reduced HRV with home monitoring systems may lead to improved outcomes in high-risk infants withcomplex congenital heart disease. Home ECG monitoring is a valid method to obtain HRV data.

Reduced heart rate variability (HRV), anindicator of autonomic nervous systemdysfunction, can be used to identify critically ill patients at higher risk for adverse outcomes.Limited data exists on HRV in children withcardiac disease. Early detection of reduced HRV with home monitoring systems may lead to improved outcomes in high-risk infants withcomplex congenital heart disease. Home ECG monitoring is a valid method to obtain HRV data.

Reduced heart rate variability (HRV), an indicator of autonomic nervous system dysfunction, can be used to identify critically ill patients at higher risk for adverse outcomes. Limited data exists on HRV in children with cardiac disease. Early detection of reduced HRV with home monitoring systems may lead to improved outcomes in high-risk infants with complex congenital heart disease.

Pulse oximetry oxygen saturation (SpO ₂ )-based critical congenital heart disease (CCHD) screening is effective in detection of cyanotic heart lesions. We report a full-term male infant with normal perfusion who had passed the CCHD screening at approximately 24 hours after birth with preductal SpO ₂ of 99% and postductal SpO ₂ of 97%. Detection of a loud systolic cardiac murmur before discharge led to the diagnosis of pulmonary atresia (PA) with ventricular septal defect (PA-VSD) by echocardiogram. The infant was transferred to a tertiary care center after initiation of prostaglandin E1 (PGE1) therapy. Throughout the initial course, he was breathing comfortably without respiratory distress or desaturations on pulse oximetry. We believe that this is the first documented report of PA missed by CCHD screening. Thorough and serial clinical examinations of the newborn infant proved vital in the timely diagnosis of this critical disease. We review the hemodynamics and the recent literature evaluating utility of CCHD screening in the diagnosis of PA-VSD. Pulse oximetry-based CCHD screening should be considered a tool to enhance CCHD detection with an emphasis on detailed serial physical examinations in newborn infants.

Objective

We evaluated first screen pass rate for two pass thresholds for critical congenital heart disease (CCHD) oxygen saturation (SpO₂) screening at higher altitude.

Study design

A retrospective cohort of 948 newborns underwent CCHD screening near sea-level (n = 463) vs 6250 ft altitude (n = 485) over 3 years. Standard SpO₂ pass threshold ≥95% and lower SpO₂ pass threshold ≥93% (high-altitude screen) were applied to first measurements to compare pass frequencies.

Results

The median SpO₂ was lower in high-altitude newborns (96% vs 99%-p < 0.001). The high-altitude newborns passed the AAP algorithm first screen less often (89.3% vs 99.6%-p < 0.001). With the high-altitude algorithm, 98% of high-altitude newborns passed the first screen.

Conclusion

Lowering the SpO₂ pass threshold by 2% at >6000 ft, significantly increased first screen pass rate. Adjustments for altitude may reduce nursing time to conduct repeat measurements and prevent transfers for echocardiograms. Larger studies are necessary to assess impact on false negatives.

Objective

Assess the quality of critical congenital heart disease (CCHD) screening data reports in California, where CCHD screening is not mandatory but reporting is.

Study design

Retrospective review of California hospital-level CCHD screening data to evaluate data reliability and adherence to state screening and reporting recommendations. Data were evaluated for internal consistency and compared to two databases.

Results

Over one-third of hospitals did not submit data. Only 70.7% of the Vital Records live births were reported in CCHD screening data. Only 46% of reporting hospitals submitted data with matching numbers of completed screens and results, and 22% matched their respective live births in a second database.

Conclusion

CCHD data reporting in California is incomplete, which may miss 359 CCHD cases/year from non-reporting. Data inconsistencies may miss additional cases. Mandatory screening, reporting, and improvements in data reliability are urgently needed to inform screening modifications and enhance timely detection and disease surveillance.

Objective

To obtain preliminary validity data for a hypoxemic respiratory failure/pulmonary hypertension (HRF/PH) score for classifying persistent pulmonary hypertension of the newborn (PPHN).

Study design

Retrospective chart review of 100 consecutive neonates admitted to a Children's hospital from 2016-2021 with PPHN, gestational age ≥34 weeks, and echocardiograms in the first week. We assessed the correlation between HRF/PH score and short-term outcomes using linear and logistic regressions.

Results

HRF/PH scores ranged 2-12 (mean 8.5), and were classified mild (0-5), moderate (6-10), and severe (11-15), with 6%, 77% and 17% infants in respective categories. HRF/PH score category correlated with invasive ventilation, nitric oxide, high frequency ventilation, vasoactive infusions, extracorporeal life support and death. HRF/PH score category did not correlate with duration of support or length of stay.

Conclusion

The HRF/PH score offers a promising representation of disease severity for PPHN. The tool requires further validation in prospective studies and evaluation for long-term outcomes.

Convulsive seizures account for 15% of pediatric air transports. We evaluated seizure treatment received in community hospital emergency departments among transported patients for adherence to recommended management. This study was a retrospective cohort study of children transported for an acute seizure to a tertiary pediatric hospital from 2010 to 2013. Seizure treatment was evaluated for adherence to recommended management. The primary outcome was intubation. Among 126 events, 61% did not receive recommended acute treatment. The most common deviation from recommended care was administration of >2 benzodiazepine doses. Lack of adherence to recommended care was associated with a greater than twofold increased risk of intubation (relative risk 2.4; 95% confidence interval, 1.4-4.13) and 1.5-fold increased risk of admission to the ICU (relative risk 1.65; 95% confidence interval, 1.24-2.16). Duration of ventilation was commonly <24 hours (87%) for patients who did or did not receive recommended acute seizure care. Among events treated initially with a benzodiazepine, only 32% received a recommended weight-based dosage, and underdosing was most common. Adherence to evidence-based recommended acute seizure treatment during initial care of pediatric patients using medical air transportation was poor. Intubation was more common when patients did not receive recommended acute seizure care. Educational efforts with a sustained quality focus should be directed to increase adherence to appropriate pediatric seizure treatment of children in community emergency departments.

UC Davis Health has a "Blackout Flag" policy (1190) that is used in trauma care settings to restrict visitors from the patient. The policy's intended goal is to protect patients from potential harmful visitors, especially in ongoing investigations. This policy may impair history taking and well-informed treatments because it restricts knowledge provided by visitors and loved ones. Often placed by police, this policy may be disproportionally applied to patients from marginalized backgrounds and may perpetuate medical distrust.