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Multidisciplinary bronchopulmonary dysplasia (BPD) programs provide improved and consistent medical management, care of the developing infant, family support, and smoother transitions in care resulting in improved survival, pulmonary, and extra-pulmonary outcomes. This review summarizes the benefits of interdisciplinary BPD management, as well as strategies for initial programmatic development, program growth, and maintenance at centers across the United States factoring in institutional, provider, and parent reported goals that were derived from a consensus conference on BPD management.
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OBJECTIVE: To develop a consensus guideline to meet nutritional challenges faced by infants with congenital diaphragmatic hernia (CDH). STUDY DESIGN: The CDH Focus Group utilized a modified Delphi method to develop these clinical consensus guidelines (CCG). Topic leaders drafted recommendations after literature review and group discussion. Each recommendation was sent to focus group members via a REDCap survey tool, and members scored on a Likert scale of 0-100. A score of > 85 with no more than 25% outliers was designated a priori as demonstrating consensus among the group. RESULTS: In the first survey 24/25 recommendations received a median score > 90 and after discussion and second round of surveys all 25 recommendations received a median score of 100. CONCLUSIONS: We present a consensus evidence-based framework for managing parenteral and enteral nutrition, somatic growth, gastroesophageal reflux disease, chylothorax, and long-term follow-up of infants with CDH.
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Consenso , Técnica Delphi , Hernias Diafragmáticas Congénitas , Humanos , Hernias Diafragmáticas Congénitas/terapia , Recién Nacido , Lactante , Reflujo Gastroesofágico/terapia , Nutrición Enteral , Nutrición Parenteral , Quilotórax/terapia , Alta del PacienteRESUMEN
Evidence on effectiveness and safety of sirolimus in congenital vascular anomalies in infancy is lacking. We aim to systematically review the efficacy and safety of sirolimus in treating congenital VA in infancy. We searched for and included all studies evaluating sirolimus for VA in the first year of life. The primary outcome was effectiveness. The secondary outcome was safety. We included 84 case series and reports (172 participants). Sirolimus decreased the size of the VA in >50% of participants, most of whom had minor transient side effects, and 27% had no adverse effects at all. When categorized by age (<1 month, 1-5 months and 6-12 months), the effectiveness was similar in all age groups. Available evidence suggests that sirolimus is effective and well tolerated. The effectiveness of sirolimus should be evaluated in a well-designed randomized controlled or observational studies.
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Sirolimus , Malformaciones Vasculares , Humanos , Sirolimus/uso terapéutico , Sirolimus/efectos adversos , Lactante , Malformaciones Vasculares/tratamiento farmacológico , Recién NacidoRESUMEN
BACKGROUND: To determine variables associated with outcomes in infants with intestinal failure (IF) and ostomy following reanastomosis (RA). METHODS: A single-center, descriptive cohort study of 120 infants with IF and a stoma from January 2011 to December 2020 with subsequent RA during initial hospitalization. The primary outcome was achievement of enteral autonomy (EA) following RA. Other outcomes were duration of hospital stay, and mortality. Penalized logistic regression and linear regression were used for data analysis. RESULTS: The median gestational age was 26 weeks, and the median birth weight was 890 g. Three infants died. The median duration between ostomy creation and RA was 80 days (interquartile range; 62.5, 100.5). For each additional day of discontinuity, the odds of EA decreased by 2% (odds ratio [OR] = 0.980; 95% confidence interval [CI]: 0.962, 0.999; P = 0.038), and death increased by 4.2% (OR = 1.042; 95% CI: 1.010, 1.075; P = 0.009). For each additional mL/kg/day of enteral feeds at RA, the odds of EA increased by 7.5% (OR = 1.075; 95% CI: 1.027, 1.126, P = 0.002) and duration of hospital stay decreased by 0.35 days (slope coefficient = -0.351; 95% CI: -0.540, -0.163; P < 0.001). CONCLUSION: Shorter duration of intestinal discontinuity and enteral nutrition before RA could positively influence EA and duration of stay in infants with IF and ostomy following RA.
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Insuficiencia Intestinal , Estomía , Lactante , Humanos , Estudios de Cohortes , Intestinos/cirugía , Peso al NacerRESUMEN
Infants born with congenital diaphragmatic hernia have high mortality and morbidity and require coordinated multidisciplinary care for optimal outcomes. Over the past several decades numerous articles have been published on the technical aspects of the care of these patients demonstrating both the variation in management across institutions as well as the desirability and need for standardization of care. Unfortunately, none have focused on the organization of care for CDH patients encompassing the range from early prenatal diagnosis to long-term postnatal care. However, to achieve optimal care and optimal outcomes, it is important to not only have excellent technical surgical and medical care but also to have an organized, systematic, and purposefully designed program for the delivery of healthcare to infants with this condition. In this article, based on our experience and drawing on general principles of building clinical programs, we describe the important elements of an ideal CDH program.
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Hernias Diafragmáticas Congénitas , Lactante , Embarazo , Femenino , Humanos , Hernias Diafragmáticas Congénitas/diagnóstico por imagen , Hernias Diafragmáticas Congénitas/cirugía , Diagnóstico Prenatal , Estudios RetrospectivosRESUMEN
BACKGROUND: Echocardiography is the gold standard for the diagnosis hemodynamically significant-patent ductus arteriosus (hs-PDA). It requires trained personnel and is not readily available. Urinary biomarkers can be used as an adjunct. OBJECTIVE: The objective of this study was to systematically review the diagnostic accuracy of urinary N terminal pro-B type natriuretic peptides (NT-proBNP) for hs-PDA in preterm neonates. METHODS: We included studies that evaluated urinary NT-proBNP and urinary NT-proBNP/creatinine ratio (index tests) in preterm neonates with hs-PDA (participants) in comparison with echocardiogram (reference standard). Methodological quality and certainty of evidence were assessed using Quality Assessment of Diagnostic-Accuracy Studies (QUADAS-2) and Grading of Recommendations Assessment, Development and Evaluation (GRADE), respectively. RESULTS: Low quality of evidence suggests that urinary NT-proBNP has modest sensitivity and specificity for the diagnosis of a hs-PDA, with variation in accuracy based on assay and patient characteristics. CONCLUSION: Urinary NT-proBNP assays must be locally validated for specific patient populations and further studies to support its use must be performed.
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Conducto Arterioso Permeable , Recien Nacido Prematuro , Lactante , Humanos , Recién Nacido , Conducto Arterioso Permeable/diagnóstico por imagen , Péptido Natriurético Encefálico , Fragmentos de Péptidos , BiomarcadoresRESUMEN
OBJECTIVE: Our objective was to investigate the correlation of Oxygen Saturation Index (OSI) with Oxygenation Index (OI) and determine OSImax values that could predict need for ECMO and death in Congenital Diaphragmatic Hernia (CDH). STUDY DESIGN: This is a retrospective cohort study of infants with CDH admitted to a tertiary level VI NICU. Pearson's correlation coefficient and simple linear regression analysis were used to investigate the OSI: OI correlation, and logistic regression analysis to investigate OSImax values that predicted need for ECMO and death. RESULTS: Among the 180 infants, OSImax value of >13 at 6 h of life (HOL) best predicted need for ECMO and death. There was a strong correlation between OSI: OI paired values (r = 0.876, p < 0.001). The linear regression equation was OI = -2.4 + 2.4(OSI). CONCLUSION: OSI could be used as a valuable adjunct to OI in the clinical management of newborn infants with CDH.
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Oxigenación por Membrana Extracorpórea , Hernias Diafragmáticas Congénitas , Recién Nacido , Lactante , Humanos , Hernias Diafragmáticas Congénitas/terapia , Estudios Retrospectivos , Saturación de Oxígeno , Análisis de los Gases de la Sangre , OxígenoRESUMEN
BACKGROUND: Platelet transfusions are routinely administered to neonates in intensive care units when there are concerns of bleeding, including high-risk situations like Extracorporeal Membrane Oxygenation (ECMO). Most platelets in ICUs are transfused prophylactically for thrombocytopenia based solely on the platelet count. Platelet Mass Index (PMI) has been proposed as an alternative to platelet count (PC) as a transfusion trigger. The objective of this study was to determine the relationship between PMI and platelet-specific maximal clot firmness (PMCF) in Rotational thromboelastometry (ROTEM), which gives an indication of platelet contribution to clot firmness and to investigate whether PMI may be a better choice as a trigger for platelet transfusions than PC. METHODS: Retrospective review of medical records of neonates with congenital heart disease placed on ECMO support in the cardiovascular intensive care unit (CVICU) from 2015 to 2018 was conducted. Platelet count (PC), platelet mean volume (PMV), ROTEM parameters along with demographic data including gestation age, birth weight, gender and survival were collected. Mixed effects linear models with a first order autoregressive covariance structure were used to assess the associations of PMI, PC, and MPV against PMCF. In addition, generalized estimating equations with a first order auto-regressive covariance structure were used to compare odds of transfusion using PC versus PMI triggers. RESULTS: A total of 92 tests on consecutive days were obtained for 12 ECMO patients (5 male, GA = 38.1 ± 1.6 weeks, BW = 3.1 ± 0.4 kgs, mean ± SD). A variation of 40.1% in PMCF was explained by platelet count (p < 0.001) while 38.5% of the variation in PMCF was explained by PMI (p < 0.001). If the platelet transfusion trigger was PC < 100 x 103 platelets/µL vs. PMI < 800. Using the PC trigger yielded significantly higher odds of transfusion compared to the PMI trigger (odds ratio = 1.31, 95% confidence interval: 1.18 - 1.45, p < 0.001). CONCLUSIONS: While our study failed to demonstrate a superior correlation of PMI with PMCF than PC, our study did reveal that using PMI as transfusion trigger would result in significantly less platelet transfusions, when compared with the current practice of using PC as a trigger.
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Oxigenación por Membrana Extracorpórea , Trombocitopenia , Recién Nacido , Humanos , Masculino , Recuento de Plaquetas , Oxigenación por Membrana Extracorpórea/efectos adversos , Trombocitopenia/terapia , Transfusión Sanguínea , Plaquetas , Transfusión de Plaquetas/efectos adversosRESUMEN
BACKGROUND: Establishment of a secure airway is a critical part of neonatal resuscitation in the delivery room and the neonatal intensive care unit. Videolaryngoscopy has the potential to facilitate successful endotracheal intubation, and decrease adverse consequences of a delay in airway stabilization. Videolaryngoscopy may enhance visualization of the glottis and intubation success in neonates. This is an update of a review first published in 2015, and updated in 2018. OBJECTIVES: To determine the effectiveness and safety of videolaryngoscopy compared to direct laryngoscopy in decreasing the time and attempts required for endotracheal intubation and increasing the success rate on first intubation attempt in neonates (0 to 28 days of age). SEARCH METHODS: In November 2022, we updated the search for trials evaluating videolaryngoscopy for neonatal endotracheal intubation in CENTRAL, MEDLINE, Embase, CINAHL, and BIOSIS. We also searched abstracts of the Pediatric Academic Societies, clinical trials registries (www. CLINICALTRIALS: gov; www.controlled-trials.com), and reference lists of relevant studies. SELECTION CRITERIA: Randomized controlled trials (RCTs), quasi-RCTs, cluster-RCTs, or cross-over trials, in neonates (0 to 28 days of age), evaluating videolaryngoscopy with any device used for endotracheal intubation compared with direct laryngoscopy. DATA COLLECTION AND ANALYSIS: Three review authors performed data collection and analysis, as recommended by Cochrane Neonatal. Two review authors independently assessed studies identified by the search strategy for inclusion. We used the GRADE approach to assess the certainty of the evidence. MAIN RESULTS: The updated search yielded 7786 references, from which we identified five additional RCTs for inclusion, seven ongoing trials, and five studies awaiting classification. Three studies were included in the previous version of the review. For this update, we included eight studies, which provided data on 759 intubation attempts in neonates. We included neonates of either sex, who were undergoing endotracheal intubation in international hospitals. Different videolaryngoscopy devices (including C-MAC, Airtraq, and Glidescope) were used in the studies. For the primary outcomes; videolaryngoscopy may not reduce the time required for successful intubation when compared with direct laryngoscopy (mean difference [MD] 0.74, 95% confidence interval [CI] -0.19 to 1.67; 5 studies; 505 intubations; low-certainty evidence). Videolaryngoscopy may result in fewer intubation attempts (MD -0.08, 95% CI -0.15 to 0.00; 6 studies; 659 intubations; low-certainty evidence). Videolaryngoscopy may increase the success of intubation at the first attempt (risk ratio [RR] 1.24, 95% CI 1.13 to 1.37; risk difference [RD] 0.14, 95% CI 0.08 to 0.20; number needed to treat for an additional beneficial outcome [NNTB] 7, 95% CI 5 to 13; 8 studies; 759 intubation attempts; low-certainty evidence). For the secondary outcomes; the evidence is very uncertain about the effect of videolaryngoscopy on desaturation or bradycardia episodes, or both, during intubation (RR 0.94, 95% CI 0.38 to 2.30; 3 studies; 343 intubations; very-low certainty evidence). Videolaryngoscopy may result in little to no difference in the lowest oxygen saturations during intubation compared with direct laryngoscopy (MD -0.76, 95% CI -5.74 to 4.23; 2 studies; 359 intubations; low-certainty evidence). Videolaryngoscopy likely results in a slight reduction in the incidence of airway trauma during intubation attempts compared with direct laryngoscopy (RR 0.21, 95% CI 0.05 to 0.79; RD -0.04, 95% CI -0.07 to -0.01; NNTB 25, 95% CI 14 to 100; 5 studies; 467 intubations; moderate-certainty evidence). There were no data available on other adverse effects of videolaryngoscopy. We found a high risk of bias in areas of allocation concealment and performance bias in the included studies. AUTHORS' CONCLUSIONS: Videolaryngoscopy may increase the success of intubation on the first attempt and may result in fewer intubation attempts, but may not reduce the time required for successful intubation (low-certainty evidence). Videolaryngoscopy likely results in a reduced incidence of airway-related adverse effects (moderate-certainty evidence). These results suggest that videolaryngoscopy may be more effective and potentially reduce harm when compared to direct laryngoscopy for endotracheal intubation in neonates. Well-designed, adequately powered RCTS are necessary to confirm the efficacy and safety of videolaryngoscopy in neonatal intubation.
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Intubación Intratraqueal , Laringoscopía , Niño , Humanos , Recién Nacido , Unidades de Cuidado Intensivo Neonatal , Intubación Intratraqueal/efectos adversos , ResucitaciónAsunto(s)
Recien Nacido Extremadamente Prematuro , Resucitación , Recién Nacido , Humanos , CapnografíaRESUMEN
BACKGROUND: Prognostication of mortality and decision to offer extracorporeal membrane oxygenation (ECMO) treatment in infants with congenital diaphragmatic hernia (CDH) can inform clinical management. OBJECTIVE: To summarise the prognostic value of echocardiography in infants with CDH. METHODS: Electronic databases Ovid MEDLINE, Embase, Scopus, CINAHL, the Cochrane Library and conference proceedings up to July 2022 were searched. Studies evaluating the prognostic performance of echocardiographic parameters in newborn infants were included. Risk of bias and applicability were assessed using the Quality Assessment of Prognostic Studies tool. We used a random-effect model for meta-analysis to compute mean differences (MDs) for continuous outcomes and relative risk (RR) for binary outcomes with 95% CIs. Our primary outcome was mortality; secondary outcomes were need for ECMO, duration of ventilation, length of stay, and need for oxygen and/or inhaled nitric oxide. RESULTS: Twenty-six studies were included that were of acceptable methodological quality. Increased diameters of the right and left pulmonary arteries at birth (mm), MD 0.95 (95% CI 0.45 and 1.46) and MD 0.79 (95% CI 0.58 to 0.99), respectively) were associated with survival. Left ventricular (LV) dysfunction, RR 2.40, (95% CI 1.98 to 2.91), right ventricular (RV) dysfunction, RR 1.83 (95% CI 1.29 to 2.60) and severe pulmonary hypertension (PH), RR 1.69, (95% CI 1.53 to 1.86) were associated with mortality. Left and RV dysfunctions, RR 3.30 (95% CI 2.19 to 4.98) and RR 2.16 (95% CI 1.85 to 2.52), respectively, significantly predicted decision to offer ECMO treatment. Limitations are lack of consensus on what parameter is optimal and standardisation of echo assessments. CONCLUSIONS: LV and RV dysfunctions, PH and pulmonary artery diameter are useful prognostic factors among patients with CDH.
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Hernias Diafragmáticas Congénitas , Hipertensión Pulmonar , Recién Nacido , Lactante , Humanos , Hernias Diafragmáticas Congénitas/diagnóstico por imagen , Hernias Diafragmáticas Congénitas/terapia , Hernias Diafragmáticas Congénitas/complicaciones , Pronóstico , Ecocardiografía , Pulmón , Hipertensión Pulmonar/diagnóstico por imagen , Hipertensión Pulmonar/complicaciones , Estudios RetrospectivosRESUMEN
INTRODUCTION: The aim of the study was to characterize the changes in fetal lung volume following fetoscopic endoluminal tracheal occlusion (FETO) that are associated with infant survival and need for extracorporeal membrane oxygenation (ECMO) in congenital diaphragmatic hernia (CDH). METHODS: Fetuses with CDH who underwent FETO at a single institution were included. CDH cases were reclassified by MRI metrics [observed-to-expected total lung volume (O/E TLV) and percent liver herniation]. The percent changes of MRI metrics after FETO were calculated. ROC-derived cutoffs of these changes were derived to predict infant survival to discharge. Regression analyses were done to determine the association between these cutoffs with infant survival and ECMO need, adjusted for site of CDH, gestational age at delivery, fetal sex, and CDH severity. RESULTS: Thirty CDH cases were included. ROC analysis demonstrated that post-FETO increases in O/E TLV had an area under the curve of 0.74 (p = 0.035) for the prediction of survival to hospital discharge; a cutoff of less than 10% was selected. Fetuses with a <10% post-FETO increase in O/E TLV had lower survival to hospital discharge [44.8% vs. 91.7%; p = 0.018] and higher ECMO use [61.1% vs. 16.7%; p = 0.026] compared to those with an O/E TLV increase ≥10%. Similar results were observed when the analyses were restricted to left-sided CDH cases. A post-FETO <10% increase in O/E TLV was independently associated with lower survival at hospital discharge (aOR: 0.073, 95% CI: 0.008-0.689; p = 0.022) and at 12 months of age (aOR: 0.091, 95% CI: 0.01-0.825; p = 0.036) as well as with higher ECMO use (aOR: 7.88, 95% CI: 1.31-47.04; p = 0.024). CONCLUSION: Fetuses with less than 10% increase in O/E TLV following the FETO procedure are at increased risk for requiring ECMO and for death in the postnatal period when adjusted for gestational age at delivery, CDH severity, and other confounders.
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Obstrucción de las Vías Aéreas , Hernias Diafragmáticas Congénitas , Embarazo , Lactante , Femenino , Humanos , Hernias Diafragmáticas Congénitas/diagnóstico por imagen , Hernias Diafragmáticas Congénitas/cirugía , Hernias Diafragmáticas Congénitas/complicaciones , Fetoscopía/métodos , Pulmón , Mediciones del Volumen Pulmonar/métodos , Atención Prenatal , Obstrucción de las Vías Aéreas/complicaciones , Tráquea/diagnóstico por imagen , Tráquea/cirugía , Ultrasonografía PrenatalRESUMEN
Although used commonly, ability of inhaled nitric oxide (iNO) to improve outcomes in infants with congenital diaphragmatic hernia (CDH) who receive extracorporeal life support (ECLS) remains controversial. We sought to determine the association between pre-ECLS use of iNO and mortality in infants with CDH from the Extracorporeal Life Support Organization (ELSO) Registry. Neonates who underwent ECLS for CDH were identified from the ELSO Registry from 2009 to 2019. Patients were categorized into those treated with iNO versus not prior to initiating ECLS. Patients were then matched 1:1 for case-mix based on pre-ECLS covariates using the propensity score (PS) for iNO treatment. The matched groups were compared for mortality. The matched cohorts were also compared for ELSO-defined systems-based complications as secondary outcomes. There were a total of 3,041 infants with an overall mortality of 52.2% and a pre-ECLS iNO use rate of 84.8%. With 1:1 matching, there were 461 infants with iNO use and 461 without iNO use. Following matching, use of iNO was not associated with a difference in mortality (odds ratio [OR] = 0.805; 95% confidence interval [CI], 0.621-1.042; p = 0.114). Results were similar in unadjusted analyses, and after controlling for covariates in the full cohort of patients and in the 1:1 matched data. Patients receiving iNO had significantly higher odds of renal complications (OR = 1.516; 95% CI, 1.141-2.014; p = 0.004), but no other significant differences were observed among secondary outcomes. ECLS use of iNO in CDH patients was not associated with a difference in mortality. Future randomized controlled studies are needed to delineate the utility of iNO in CDH patients.
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Oxigenación por Membrana Extracorpórea , Hernias Diafragmáticas Congénitas , Recién Nacido , Lactante , Humanos , Óxido Nítrico , Hernias Diafragmáticas Congénitas/terapia , Puntaje de Propensión , Oxigenación por Membrana Extracorpórea/métodos , Administración por Inhalación , Estudios RetrospectivosRESUMEN
Background: Neonates 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. Methods: This 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. Results: We 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. Conclusion: This 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.
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BACKGROUND: The definition of systemic hypertension in the neonatal population remains elusive given the variability of normative blood pressure measurements. Inadequate literature exists about incidence, therapy and long-term management of systemic hypertension in neonates. We aimed to describe the current incidence of neonatal systemic hypertension, differences in demographic and risk factors data. METHODS: Retrospective cohort of neonates (≤28 days) admitted to a neonatal intensive care unit participating in Pediatric Health Information System (PHIS) between Jan 2010 and December 2020 with an ICD 9/10 code for hypertension. Patients were excluded if they had congenital heart disease lesions that might contribute to systemic hypertension or had incomplete data. RESULTS: There were a total of 2494 hypertensive patients among the 432,367 NICU patients meeting the study inclusion criteria, with an incidence of 0.6%. Patients with hypertension were significantly more likely to die before discharge compared to patients without HTN (8.4% versus 3.8%, respectively, p < 0.001). Of the 2494, 52.8% received at least one antihypertensive agent, with hydralazine being the most prescribed agent (29.7%). CONCLUSION: Diagnosis of Systemic hypertension continues to increase in the neonatal population, despite absence of well-defined criteria necessitating targeted medical management. A consensus guideline which addresses this very important condition is beneficial.