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This survey substantiates the variability of immunizations practices in the NICU and identifies reasons for this variability. Future studies should inform better practice guidance for immunization of preterm NICU patients based on vaccine safety and effectiveness.

Congenital diaphragmatic hernia (CDH) is a rare congenital anomaly, whose presentation is complicated by pulmonary hypertension (PH), pulmonary hypoplasia, and myocardial dysfunction, each of which have significant impact on short-term clinical management and long-term outcomes. Despite many advances in therapy and surgical technique, optimal CDH management remains a topic of debate, due to the variable presentation, complex pathophysiology, and continued impact on morbidity and mortality. One of the more recent management strategies is the use of prostaglandin E1 (PGE1) infusion in the management of PH associated with CDH. PGE1 is widely used in the NICU in critical congenital cardiac disease to maintain ductal patency and facilitate pulmonary and systemic blood flow. In a related paradigm, PGE1 infusion has been used in situations of supra-systemic right ventricular pressures, including CDH, with the therapeutic intent to maintain ductal patency as a “pressure relief valve” to reduce the effective afterload on the right ventricle (RV), optimize cardiac function and support pulmonary and systemic blood flow. This paper reviews the current evidence for use of PGE1 in the CDH population and the opportunities for future investigations.

The ductus arteriosus (DA) is a unique fetal vascular shunt, which allows blood to bypass the developing lungs in utero. After birth, changes in complex signaling pathways lead to constriction and permanent closure of the DA. The persistent patency of the DA (PDA) is a common disorder in preterm infants, yet the underlying causes of PDA are not fully defined. Although limits on the availability of human DA tissues prevent comprehensive studies on the mechanisms of DA function, mouse models have been developed that reveal critical pathways in DA regulation. Over 20 different transgenic models of PDA in mice have been described, with implications for human DA biology. Similarly, we enumerate 224 human single-gene syndromes that are associated with PDA, including a small subset that consistently feature PDA as a prominent phenotype. Comparison and functional analyses of these genes provide insight into DA development and identify key regulatory pathways that may serve as potential therapeutic targets for the management of PDA.

BackgroundNeonates with congenital diaphragmatic hernia (CDH) have varying degrees of pulmonary hypoplasia, pulmonary hypertension (PH) and cardiac dysfunction. These neonates frequently require vasoactive support and are at high risk for mortality and morbidity, including prolonged ventilator support, need for extracorporeal membrane oxygenation (ECMO), prolonged length of stay, and need for tracheostomy. However, identifying which infants are at increased risk can be challenging. In this study, we sought to investigate the utility of the inotropic score (IS) and vasoactive inotropic score (VIS) as tools to predict significant clinical outcomes and overall survival in patients with CDH. Additionally, we evaluated the correlation between IS/VIS and postnatal echocardiographic variables.MethodsThis was a retrospective chart review of 57 patients with CDH whose postnatal care was based on a standardized institutional protocol. We calculated the IS/VIS at 6-, 12-, 24-, 48 hours of life (HOL), on the day of CDH repair and 24- and 48 hours after surgical repair. The association of these scores with postnatal echocardiographic markers was analyzed using Pearson's correlation and linear regression, while logistic regression was used for binary outcomes, and Cox proportional hazards regression was used to assess associations with survival.ResultsWe found that every one-unit increase in IS/VIS at 6 HOL was associated with 13% increase in the odds of ECMO (p = 0.034) and 10.1% increase in risk of death (p = 0.021). An increase in IS/VIS at 12-, 24- and 48-HOL was associated with posterior septal bowing in the first postnatal echocardiogram (p < 0.05 for all). Additionally, we noted an inverse relationship between IS (r = −0.281, p = 0.036) and VIS (r = −0.288, p = 0.031) on the day of repair and left ventricle (LV) systolic function in first postnatal echocardiogram. Increase in IS (r = −0.307, p = 0.024) and VIS (r = −0.285, p = 0.037) on the day of repair was associated with decreased LV function on the post-repair echocardiogram.ConclusionThis retrospective study showed a significant association between IS/VIS obtained at various time points with clinical outcomes and echocardiographic findings in CDH, which could be used to guide prognosis and management in this patient population.