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ObjectiveTo determine the incidence, risk factors, and outcomes of pulmonary hypertension (PH) in the pediatric intensive care unit (PICU) after pediatric hematopoietic stem cell transplant (HCT).MethodsThis was a retrospective study of pediatric patients who underwent allogeneic HCT between January 2008-December 2014 at a center contributing to the Center for International Blood and Marrow Transplant Research data registry. Incidence of PH was assessed from PICU diagnostic codes from records merged from the Virtual Pediatric Systems database. Regression and survival analyses identified factors associated with post-HCT PH. Additional post-HCT morbidities and survival after PH were also assessed.ResultsAmong 6,995 HCT recipients, there were 29 cases of PH, a cumulative incidence of 0.42% (95% CI 0.27%-0.57%) at 60 months post-HCT. In the sub-cohort of 1,067 patients requiring intensive care after HCT, this accounted for a PH prevalence of 2.72% (95% CI 1.74–3.69%). There was an increased risk of developing PH associated with Black/African American race, metabolic disorders, partially HLA-matched or cord blood allografts, graft-versus-host prophylaxis regimen, and lower pre-HCT functional status. Patients who developed PH had significant PICU comorbidities including heart failure, pulmonary hemorrhage, respiratory failure, renal failure, and infections. Survival at 6 months after diagnosis of post-HCT PH was 51.7% (95% CI 32.5%-67.9%).ConclusionsPH is a rare but serious complication in the pediatric post-HCT population. A significant burden of additional comorbidities, procedural interventions, and risk of mortality is associated with its development. Close monitoring and prompt intervention for this severe complication are necessary in this vulnerable population.

Objective: To investigate the complex interplay between fertility treatment, multiple gestations, and prematurity. Design: Retrospective cohort study linking the national Center for Disease Control and Prevention infant birth and death data from 2014 to 2018. Setting: National database from Center of Disease Control and Prevention. Patients: In total, 19,454,155 live-born infants with gestational ages 22–44 weeks, 114,645 infants born using non IVF fertility treatment (NIFT), and 179,960 via assisted reproductive technology (ART). Intervention: Noninvasive fertility treatment or ART vs. spontaneously conceived pregnancies. Main Outcome Measures: The main outcome assessed was prematurity. Formal mediation analysis was conducted to calculate the percentage mediated by multiple gestations. Results: Newborns born using NIFT or ART compared with those with no fertility treatment had a higher incidence of multiple gestation (no fertility treatment = 3.0%; NIFT = 24.7%; ART = 32.7%; P

Abstract Inhaled iloprost (iILO) has shown efficacy in treating patients with hypoxic lung disease and pulmonary hypertension, inducing selective pulmonary vasodilation and improvement in oxygenation. However, its short elimination half‐life of 20–30 min necessitates frequent intermittent dosing (6–9 times per day). Thus, the administration of iILO via continuous nebulization represents an appealing method of drug delivery in the hospital setting. The objectives are: (1) describe our continuous iILO delivery methodology and safety profile in mechanically ventilated pediatric pulmonary hypertension patients; and (2) characterize the initial response of iILO in these pediatric patients currently receiving iNO. Continuous iILO was delivered and well tolerated (median 6 days; range 1–94) via tracheostomy or endotracheal tube using the Aerogen® mesh nebulizer system coupled with a Medfusion® 400 syringe pump. No adverse events or delivery malfunctions were reported. Initiation of iILO resulted in an increase in oxygen saturation from 81.4 ± 8.6 to 90.8 ± 4.1%, p 1 day resulted in a higher response rate to iILO (as defined as a ≥ 4% increase in saturations) compared to those receiving iNO

ObjectivesIn fetuses with left-sided congenital diaphragmatic hernia (CDH), left heart structures may appear small, but usually normalize after birth in the absence of structural cardiac anomalies. To decrease the possibility of an erroneous diagnosis of structural heart disease, we identify normal values for left heart structures in the presence of left CDH and secondarily investigate the relationship of left heart size and survival to neonatal hospital discharge.MethodsLeft heart structures [mitral valve (MV) and aortic valve (AoV) annulus diameter, left ventricle (LV) length and width] were measured by fetal echocardiogram in fetuses with left CDH and no congenital heart disease. We generated linear regression models to establish the relationship of gestational age for each left heart structure using data from fetuses who survived after birth. We calculated z-scores (normalized to gestational age), and assessed the relationship of survival to the size of each structure.ResultsOne hundred forty-two fetuses underwent fetal echocardiogram (median 25 weeks' gestation, IQR 23, 27 weeks). Left heart structures were deemed small when using published normative data from unaffected fetuses (z-scores: MV −1.09 ± 1.35, AoV −2.12 ± 1.16, LV length −1.36 ± 1.24, LV width −4.79 ± 0.79). CDH-specific models derived from log-transformed values yielded left-shifted distributions, reflecting the small structures (mean z-score lower by: MV 0.99 ± 0.30, AoV 2.04 ± 0.38, LV length 1.30 ± 0.36, and LV width 4.69 ± 0.28; p

Abstract We present a case of a late preterm infant placed on extracorporeal life support in the first day of life for persistent pulmonary hypertension of the newborn. Developmental arrest, pulmonary vascular hypertensive changes, and pulmonary interstitial glycogenosis were present on lung biopsy at 7 weeks of age. Pulmonary hypertension has persisted through childhood. Genetic testing at 8 years identified a novel mutation in TBX4.

Background Infants with critical congenital heart disease (CCHD) are more likely to be small for gestational age (SGA) or born to mothers with maternal placental syndrome. The objective of this study was to investigate the relationship between maternal placental syndrome, SGA, and gestational age (GA) on 1‐year mortality in infants with CCHD. Methods and Results In a population‐based administrative database of all live‐born infants in California (2007–2012) we identified all infants with CCHD without chromosomal anomalies. Our primary predictor was an impaired fetal environment (IFE), defined as presence of maternal placental syndrome or SGA. We calculated hazard ratios to quantify the association between different components of IFE and 1‐year mortality and conducted a causal mediation analysis to assess GA at birth as a mediator. We identified 6863 infants with CCHD. IFE was present in 25.1%. Infants with IFE were more likely to die than infants without IFE (16.6% versus 11.1%; hazard ratios 1.55, 95% CI 1.34–1.78). Only SGA (hazard ratios 1.76, 95% CI 1.50–2.05) and placental abruption (hazard ratios 1.70, 95% CI 1.17–2.48) were significantly associated with mortality; preeclampsia and gestational hypertension had no significant association with mortality. The mediation analysis showed that 32.8% (95% CI 24.9–47.0%) of the relationship between IFE and mortality is mediated through GA. Conclusions IFE is a significant contributor to outcomes in the CCHD population. SGA and placental abruption are the main drivers of postnatal mortality while other maternal placental syndrome components had much less of an impact. Only one third of the effect between IFE and mortality is mediated through GA.

Background Infants with critical congenital heart disease (CCHD) are more likely to be small for gestational age (GA). It is unclear how this affects mortality. The authors investigated the effect of birth weight Z score on 1‐year mortality separately in preterm (GA

Despite advances in the diagnosis and management of pediatric pulmonary hypertension (PH), children with PH represent a growing inpatient population with significant morbidity and mortality. To date, no studies have described the clinical characteristics of children with PH in the pediatric intensive care unit (PICU). A retrospective multicenter cohort study of 153 centers in the Virtual PICU Systems database who submitted data between 1 January 2009 and 31 December 2015 was performed. A total of 14,880/670,098 admissions (2.2%) with a diagnosis of PH were identified. Of these, 2190 (14.7%) had primary PH and 12,690 (85.3%) had secondary PH. Mortality for PH admissions was 6.8% compared to 2.3% in those admitted without PH (odds ratio = 3.1; 95% confidence interval = 2.9–3.4). Compared to patients admitted to the PICU without PH, those with PH were younger, had longer length of stay, higher illness severity scores, were more likely to receive invasive mechanical ventilation, cardiopulmonary resuscitation, extracorporeal membrane oxygenation, and more likely to have co-diagnoses of sepsis, heart failure, and respiratory failure. In a multivariate model, factors significantly associated with mortality for children with PH included age 16 years, invasive mechanical ventilation, and co-diagnoses of heart failure, sepsis, hemoptysis, disseminated intravascular coagulation, stroke, and multi-organ dysfunction syndrome. Despite therapeutic advances, the disease burden and mortality of children with PH remains significant. Further investigation of the risk factors associated with clinical deterioration and mortality in this population could improve the ability to prognosticate and inform clinical decision-making.