Outcomes of atrioventricular septal defects with and without down syndrome: analysis of the national inpatient database.

BACKGROUND: Controversial data exist about the impact of Down syndrome on outcomes after surgical repair of atrioventricular septal defect. AIMS: (A) assess trends and outcomes of atrioventricular septal defect with and without Down syndrome and (B) determine risk factors associated with adverse outcomes after atrioventricular septal defect repair. METHODS: We queried The National Inpatient Sample using International Classification of Disease codes for patients with atrioventricular septal defect < 1 year of age from 2000 to 2018. Patients' characteristics, co-morbidities, mortality, and healthcare utilisation were evaluated by comparing those with versus without Down syndrome. RESULTS: In total, 2,318,706 patients with CHD were examined; of them, 61,101 (2.6%) had atrioventricular septal defect. The incidence of hospitalisation in infants with atrioventricular septal defect ranged from 4.5 to 7.5% of all infants hospitalised with CHD per year. A total of 33,453 (54.7%) patients were associated with Down syndrome. Double outlet right ventricle, coarctation of the aorta, and tetralogy of Fallot were the most commonly associated with CHD in 6.9, 5.7, and 4.3% of patients, respectively. Overall atrioventricular septal defect mortality was 6.3%. Multivariate analysis revealed that prematurity, low birth weight, pulmonary hypertension, and heart block were associated with mortality. Down syndrome was associated with a higher incidence of pulmonary hypertension (4.3 versus 2.8%, p < 0.001), less arrhythmia (6.6 versus 11.2%, p < 0.001), shorter duration for mechanical ventilation, shorter hospital stay, and less perioperative mortality (2.4 versus 11.1%, p < 0.001). CONCLUSION: Trends in atrioventricular septal defect hospitalisation had been stable over time. Perioperative mortality in atrioventricular septal defect was associated with prematurity, low birth weight, pulmonary hypertension, heart block, acute kidney injury, and septicaemia. Down syndrome was present in more than half of atrioventricular septal defect patients and was associated with a higher incidence of pulmonary hypertension but less arrhythmia, lower mortality, shorter hospital stay, and less resource utilisation.

Kidney replacement therapy in neonates: utilization trends and outcomes.

BACKGROUND: We aimed to assess prevalence and clinical characteristics of newborns receiving kidney replacement therapy (KRT). METHODS: We used the National Inpatient Sample (NIS) dataset for the years 2000-2017. Newborns treated with peritoneal dialysis (PD), hemodialysis (HD), and continuous KRT (CKRT) were included. Trend analysis using the Cochran-Armitage test was used to assess prevalence over the years. RESULTS: A total of 64,532,552 hospitalized newborns were included. Among the 4281 infants treated with KRT, 2501 (58.4%) were treated with PD, 997 (23.3%) had HD, and 783 (18.3%) used CKRT. Associated diagnoses included congenital kidney anomalies (37.4% vs. 15% vs. 9.5%), urinary tract anomalies (35% vs. 12.5% vs. 6.3%), and congenital heart disease (68% vs. 25.7% vs. 72.3%). Median length of stay was longest in PD patients (39 days vs. 18 days vs. 26 days), respectively. However, cost of hospitalization was greatest in CKRT patients (US $490,916 vs. US $218,514 vs. US $621,554), respectively. In the entire cohort, 54,424 newborns had acute kidney injury (AKI); of them 16,999 (31%) died. KRT was used in 2,688 (4.9%) of infants with AKI. Over the study period, trends for utilization of PD (from 0.042 to 0.06%) and CKRT (from 0.03 to 0.21%) increased whereas the hemodialysis trend decreased (from 0.021 to 0.013%). CONCLUSIONS: Congenital heart disease (CHD) and congenital anomalies of the kidneys and urinary tract (CAKUT) are the major diagnoses in newborns receiving KRT. Utilization of PD was greater than HD and CKRT. Trends of PD and CKRT utilization increased over time. Less than 5% of infants diagnosed with AKI received KRT.

Stroke in pediatric ECMO patients: analysis of the National Inpatient Sample (NIS) database.

BACKGROUND: The rates, outcomes, and long-term trends of stroke complicating the use of extracorporeal membrane oxygenation (ECMO) have been inconsistently reported. We compared the outcomes of pediatric ECMO patients with and without stroke and described the frequency trends between 2000 and 2017. METHODS: Using the National Inpatient Sample (NIS) database, pediatric patients (age ≤18 years) who received ECMO were identified using ICD-9&10 codes. Binary, regression, and trend analyses were performed to compare patients with and without stroke. RESULTS: A total of 114,477,997 records were reviewed. Overall, 28,695 (0.025%) ECMO patients were identified of which 2982 (10.4%) had stroke, which were further classified as hemorrhagic (n = 1464), ischemic (n = 1280), or combined (n = 238). Mortality was higher in the hemorrhagic and combined groups compared to patients with ischemic stroke and patients without stroke. Length of stay (LOS) was significantly longer in stroke vs. no-stroke patients. Hypertension and septicemia were more encountered in the hemorrhagic group, whereas the combined group demonstrated higher frequency of cardiac arrest and seizures. CONCLUSIONS: Over the years, there is an apparent increase in the diagnosis of stroke. All types of stroke in ECMO patients are associated with increased LOS, although mortality is increased in hemorrhagic and combined stroke only. IMPACT: Stroke is a commonly seen complication in pediatric patients supported by ECMO. Understanding the trends will help in identifying modifiable risk factors that predict poor outcomes in this patient population.

Trends in Procedures in the Neonatal Intensive Care Unit.

OBJECTIVE: The aim of the study is to identify the rates and trends of various procedures performed on newborns. STUDY DESIGN: The Healthcare Cost and Utilization Project (HCUP) database for the years 2002 to 2015 was queried for the number of livebirths, and various procedures using International Classification of Diseases, Ninth Revision (ICD-9) codes. These were adjusted to the rate of livebirths in each particular year. A hypothetical high-volume hospital based on data from the last 5 years was used to estimate the frequency of each procedure. RESULTS: Over the study period, there was a decline in the rates of exchange transfusions and placement of arterial catheters. There was an increase in the rates of thoracentesis, abdominal paracentesis, placement of umbilical venous catheter (UVC) lines, and central lines with ultrasound or fluoroscopic guidance. No change was observed in the rates of unguided central lines, pericardiocentesis, bladder aspiration, intubations, and LP. Intubations were the most performed procedures. Placement of UVC, central venous lines (including PICCs), arterial catheters, and LP were relatively common, whereas others were rare such as pericardiocentesis and paracentesis. CONCLUSION: Some potentially lifesaving procedures are extremely rare or decreasing in incidence. There has also been an increase in utilization of fluoroscopic/ultrasound guidance for the placement of central venous catheters. KEY POINTS: · Advances in neonatal care have impacted the number of procedures performed in the NICU.. · It is unclear whether invasive procedures occur at rates sufficient for adequate training and maintenance of skills.. · Understanding the NICU procedural trends is important in designing simulation and competency-based medical education programs..

The U.S. National Trend for Retinopathy of Prematurity.

OBJECTIVE: The use of supplemental oxygen in premature infants is essential for survival. However, its use has been associated with unintended complications. The restricted use of oxygen is associated with increased mortality and necrotizing enterocolitis (NEC), whereas its liberal use is associated with increased risk for retinopathy of prematurity (ROP). Although there is no clear consensus on the acceptable oxygen saturation range, clinicians have recently become more liberal with the use of oxygen. We aim to assess (1) the national trends for ROP in very low birth weight preterm infants, and (2) the associated trends in mortality, NEC, intraventricular hemorrhage (IVH), and length of hospital stay (LOS). STUDY DESIGN: We analyzed deidentified patient data from the National Inpatient Sample (NIS) of the Healthcare Cost and Utilization Project (HCUP) from 2002 to 2017. All infants with gestational age ≤32 weeks and birth weight <1,500 g were included. Trends in ROP, severe ROP, mortality, NEC, IVH, severe IVH, and LOS were analyzed using Jonckheere-Terpstra test. RESULTS: A total of 818,945 neonates were included in the study. The overall mortality was 16.2% and the prevalence of ROP was 17.5%. There was a significant trend for increased ROP over the years (p < 0.001). Severe ROP was also significantly increased (p < 0.001). This was associated with a significant trend for increased median LOS in survived infants (p < 0.001). Mortality was significantly decreased (p < 0.001), whereas NEC and severe NEC did not change over time (p = 0.222 and p = 0.412, respectively). CONCLUSION: There is a national trend for increased ROP and severe ROP over the 16 years of the study period. This trend was associated with a significant increase in the LOS in survived infants without change in NEC. KEY POINTS: · Prevalence of ROP and severe ROP has increased in VLBW infants over the 16-year study period.. · The prevalence of NEC did not change over the same time period.. · Increased ROP and severe ROP were consistent in all three GA and BW subgroups..

Outcomes of Cardiac Arrest and Cardiopulmonary Resuscitation in Patients With Left Ventricular Assist Device; an Insight From a National Inpatient Sample.

BACKGROUND: Outcomes of patients with implanted left ventricular assist device (LVAD) implantation experiencing a cardiac arrest (CA) are not well reported. We aimed at defining the in-hospital outcomes of patients with implanted LVAD experiencing a CA. METHODS: The national inpatient sample (NIS) was queried using ICD9/ICD10 codes for patients older than 18 years with implanted LVAD and CA between 2010-2018. We excluded patients with orthotropic heart transplantation, biventricular assist device (BiVAD) implantation and do not resuscitate (DNR) status. RESULTS: A total of 93,153 hospitalisations between 2010 and 2018 with implanted LVAD were identified. Only 578 of these hospitalisations had experienced CA and of those, 173 (33%) hospitalisations underwent cardiopulmonary resuscitation (CPR). The mean age of hospitalisations that experienced a CA was 60.61±14.85 for non-survivors and 56.23±17.33 for survivors (p=0.14). The in-hospital mortality was 60.8% in hospitalisations with CA and 74.33% in hospitalisations in whom CPR was performed. In an analysis comparing survivors with non-survivors, non-survivors had more diabetes mellitus (DM) (p=0.01), and ischaemic heart disease (IHD) (p=0.04). Age, female sex, peripheral vascular disease and history of coronary artery bypass graft (CABG) were independently associated with increased mortality in our cohort. Also, ventricular tachycardia (VT) and CPR were independently associated with in-hospital mortality. During the study period, there was a significantly decreasing trend in performing CPR in LVAD hospitalisations with CA. CONCLUSION: In conclusion, age, female sex, peripheral vascular disease, history of CABG, VT and CPR were independently associated with in-hospital mortality in LVAD hospitalisations who experienced CA.

Trends and racial disparities for acute kidney injury in premature infants: the US national database.

BACKGROUND: To assess prevalence and outcomes of acute kidney injury (AKI) in very-low-birth-weight infants. METHODS: This cross-sectional study utilized the National Inpatient Sample (NIS) dataset for years 2000-2017. All premature infants with birth weight (BW) <1500g and/or gestational age (GA) ≤32 weeks were included. Analyses were conducted for overall population and two BW categories: <1000g and 1000-1499g. Adjusted odds ratios were calculated after controlling for confounding variables in logistic regression analysis. Cochrane-Armitage test was used to assess for statistically significant trends in AKI frequency over the years. RESULTS: In total, 1,311,681 hospitalized premature infants were included; 19,603 (1.5%) were diagnosed with AKI. The majority (74.3%) were BW <1000g and 63.9% ≤28 weeks gestation. Prevalence of AKI differed by ethnicity; White had significantly less AKI than Black (OR=0.79, p<0.001) and Hispanic (OR=0.83, p<0.001). AKI was significantly associated with higher mortality compared to controls (35.1 vs. 3.0%, p<0.001). AKI was associated with comorbidities such as necrotizing enterocolitis, patent ductus arteriosus, bronchopulmonary dysplasia, intraventricular hemorrhage, and septicemia. In a regression model, AKI was associated with higher mortality after controlling confounding factors (aOR=7.79, p<0.001). AKI was associated with significant increase in length of stay (p<0.001) and cost of hospitalization in survivors (p<0.001). There is a significant trend for increased AKI frequency over the years (Z score=4.33, p<0.001). CONCLUSION: AKI is associated with increased mortality and comorbidities in preterm infants, especially in infants with BW <1000g. Further studies are needed to understand precipitating factors and assess preventative measures for this serious complication.

Esophageal perforation in very low birth weight infants.

We aimed to assess the prevalence and outcomes of esophageal perforation in very low birth weight infants. This retrospective cohort study utilized the US National Inpatient Sample dataset for the years 2000 to 2017. A total of 1,755,418 very low birth weight infants were included; of them, 861 (0.05%) were diagnosed with esophageal perforation. The majority (77.9%) of infants were in the birth weight category < 1000 g and 77.7% in infants ≤ 28 weeks of gestation. The majority (73%) of infants were tracheally intubated and received mechanical ventilation; of them, 24 infants (2.8%) had tracheostomy. Mortality associated with esophageal perforation was 25.8%. Regression analysis did not show an association between esophageal perforation and increased mortality in preterm infants (aOR = 1.0, CI: 0.83-1.20, p = 0.991). Procedures encountered in these infants include thoracentesis (10.8%), laparotomy (4.1%), percutaneous abdominal drainage (4.1%), and gastrostomy tube insertion (6.2%), whereas the rest of the infants were managed conservatively. There was a significant trend for increasing prevalence of esophageal perforation over the years.Conclusion: Esophageal perforation does not independently increase the risk for mortality in very low birth weight infants. The increasing prevalence is possibly related to increased care offered to infants at limits of viability in recent years. What is Known: • Knowledge about esophageal perforation is derived from anecdotal single-center case reports. • Esophageal perforation in neonates is mostly iatrogenic. • It is considered a critical complication that is associated with high mortality. What is New: • This is the first and largest national study on prevalence of esophageal perforation in preterm infants. • Esophageal perforation does not independently increase the risk for mortality. • Septicemia and pneumothorax are frequent complications to esophageal perforation.

Survival outcomes of in-hospital cardiac arrest in pediatric patients in the USA.

We report on in-hospital cardiac arrest outcomes in the USA. The data were obtained from the National (Nationwide) Inpatient Sample datasets for the years 2000-2017, which includes data from participating hospitals in 47 US states and the District of Columbia. We included pediatric patients (< 18 years of age) with cardiac arrest, and we excluded patients with no cardiopulmonary resuscitation during the hospitalization. Primary outcome of the study was in-hospital mortality after cardiac arrest. A multivariable logistic regression was performed to identify factors associated with survival. A total of 20,654 patients were identified, and 8226 (39.82%) patients survived to discharge. The median length of stay and cost of hospitalization were significantly higher in the survivors vs. non-survivors (LOS 18 days vs. 1 day, and cost $187,434 vs. $45,811, respectively, p < 0.001). In a multivariable model, patients admitted to teaching hospitals, elective admissions, and those admitted on weekdays had higher survival (aOR=1.19, CI: 1.06-1.33; aOR=2.65, CI: 2.37-2.97; and aOR=1.17, CI: 1.07-1.27, respectively). There was no difference in mortality between patients with extracorporeal cardiopulmonary resuscitation (E-CPR) and those with conventional cardiopulmonary resuscitation. E-CPR patients were likely to have congenital heart surgery (51.0% vs. 20.8%).Conclusion: We highlighted the survival predictors in these events, which can guide future studies aimed at improving outcomes in pediatric cardiac arrest. What is Known: • In-hospital cardiac arrest occurs in 2-6% of pediatric intensive care admissions. • Cardiac arrests had a significant impact on hospital resources and a significantly high mortality rate. What is New: • Factors associated with higher survival rates in patients with cardiac arrest: admission to teaching hospitals, elective admissions, and week-day admissions. • The use of rescue extracorporeal cardiopulmonary resuscitation in refractory cardiac arrest has increased by threefold over the last two decades.

Outcomes of hypoplastic left heart syndrome: analysis of National Inpatient Sample Database 1998-2004 versus 2005-2014.

Neonates with hypoplastic left heart syndrome (HLHS) were identified from the National Inpatient Sample dataset for the years 1998-2014. These patients were stratified into two chronological groups, past group (1998-2005) and recent group (2006-2014). A total of 20,649 neonates with HLHS were identified. Of them, 9179 (44.5%) were born in the past group and 11,470 (55.5%) in the recent group. Median birth weight was significantly less in the recent group (2967 g vs. 3110 g, p = 0.005). The patients in the recent group had more patients with low birth weight ( < 2.5 kg) and prematurity (8.7% vs 7.6% and 12.7% vs. 4.3%., respectively). In addition, recent group had more comorbidities including chromosomal anomalies, total anomalous pulmonary venous return, and kidney anomalies (5.6% vs. 3.6%, 2.3% vs. 1.7%, and 5.6% vs. 3.6%, respectively, p < 0.001); these were associated with a higher rate of extracorporeal membrane oxygenation utilization (9.2% vs. 4.5%, p < 0.001). Consequently, median length of stay was longer in the recent group (8 vs. 6 days, p < 0.001).Conclusion: Despite the higher frequency of comorbidities in recent group, the mortality rates decreased by 20% (from 25.3% to 20.6%, p < 0.001). Balloon atrial septostomy was performed less frequently in the recent group (23.3% vs. 16.1%, p < 0.001).What is known:• Hypoplastic left heart syndrome has the highest mortality among congenital cardiac defects during the first year of life.• Limited studies on patients' comorbidities and mortality rates trends over last two decades.What is new:• The study utilized a national database to compare in-hospital mortality and length of stay between the two time periods 1998-2005 and 2006-2014.• The recent group had more comorbidities (prematurity, chromosomal anomalies, total anomalous pulmonary venous return, and kidney anomalies), and there was higher rate of ECMO and longer length of stay, while mortality rates decreased by 20%.

Impact of ventricular arrhythmias on outcomes in children with myocarditis.

Children affected with acute myocarditis may progress rapidly into profound ventricular dysfunction and ventricular arrhythmias. The objective of this study is to assess the impact of ventricular arrhythmias on in-hospital mortality and the use of mechanical circulatory support in patients with myocarditis. Pediatric patients (age 0-18 years) admitted with myocarditis were identified from the National Inpatient Sample dataset for the years 2002-2015. A total of 12,489 patients with myocarditis were identified. Of them, 1627 patients were with ventricular arrhythmias and 10,862 patients without ventricular arrhythmias. Mortality was higher in those with ventricular arrhythmias (19.5% vs. 2.8%, OR = 8.47; 95% CI 7.16-10.04; p < 0.001). The median length of stay and the median cost of hospitalization were higher in the ventricular arrhythmias group (9 days vs. 4 days, p < 0.001 and $121,826 vs. $37,658, p < 0.001, respectively). There was a substantial increase in the utilization of extracorporeal membrane oxygenation (ECMO) in patients with ventricular arrhythmias (25.4% vs. 2.7%, OR = 12.40; 95% CI 10.55-14.57; p < 0.001). The use of ventricular assist devices (VADs) was higher in patients with ventricular arrhythmias (4.5% vs. 1.3%, OR = 3.76; 95% CI 2.82-5.01; p < 0.001). An improvement in discharge survival was observed over the years of study in both VA and non-VA groups; associated with this decline in mortality, there was a rising trend of ECMO utilization.Conclusion: Development of ventricular arrhythmia in children with myocarditis is a strong predictor for mortality and ECMO utilization. What is Known: • The clinical presentation of pediatric myocarditis varies from no symptoms of myocardial dysfunction to a rapidly progressing severe congestive heart failure. • Little is known about the predictors of mortality in children with suspected myocarditis. What is New: • Development of ventricular arrhythmia in children with myocarditis is a strong predictor for mortality and ECMO utilization. • Improvement in discharge survival was observed over the years of study; associated with this decline in mortality, there was a rising trend of ECMO utilization.

Prevalence and Outcomes of Balloon Atrial Septostomy in Neonates With Transposition of Great Arteries.

OBJECTIVES: Transposition of the great arteries is the most common cyanotic congenital heart defect. Surgical correction usually occurs in the first week of life; presence of restrictive interatrial communication and severe hypoxemia warrants urgent intervention with balloon atrial septostomy and medical stabilization prior to surgery. The main objective of this study is to compare the characteristics, outcomes, and mortality risks in patients with transposition of the great arteries who underwent balloon atrial septostomy during their hospitalization versus transposition of the great arteries patients who have not undergone this procedure. DESIGN: Retrospective analysis of administrative data. SETTING: Data from Kids' Inpatient Database complemented with the National Inpatient Sample dataset for the years 1998-2014, this includes data from participating hospitals in 47 U.S. States and the District of Columbia. PATIENTS: Neonates admitted with transposition of the great arteries. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: A total of 17,392 neonates with diagnosis of transposition of the great arteries were captured in the databases we used. Male-to-female ratio was 2:1. The rate of balloon atrial septostomy in patients with transposition of the great arteries was 27.7% without significant change over the years. There was no significant difference in mortality between balloon atrial septostomy and no balloon atrial septostomy (6.3% vs 6.7%; p = 0.29). Neonates with balloon atrial septostomy had a two-fold increase in their length of stay compared with no balloon atrial septostomy (16 d vs 7 d; p < 0.0001). Stroke was present in 1.1% of balloon atrial septostomy group versus 0.6% in those who did not have balloon atrial septostomy (odds ratio, 1.85; 95% CI, 1.29-2.65; p < 0.0001). Extracorporeal membrane oxygenation was used more in balloon atrial septostomy group (5.1% vs 3.1%; p < 0.0001). CONCLUSIONS: There was no difference in mortality rate between balloon atrial septostomy and no balloon atrial septostomy patients. The prevalence of the diagnosis of stroke in this study was higher in patients who underwent balloon atrial septostomy. Furthermore, comparison of in-hospital mortality in balloon atrial septostomy and no balloon atrial septostomy revealed increased mortality risk in no balloon atrial septostomy patients transferred from other institution, no balloon atrial septostomy patients supported with extracorporeal membrane oxygenation, and balloon atrial septostomy patients diagnosed with stroke. Finally, length of stay and charges were higher in balloon atrial septostomy patients.

Clinical Outcomes and Risk Factors for In-Hospital Mortality in Neonates with Hypoplastic Left Heart Syndrome.

The objective of this study was to identify patient and hospitalization characteristics associated with in-hospital mortality in infants with hypoplastic left heart syndrome (HLHS). We conducted a retrospective analysis of a large administrative database, the National Inpatient Sample dataset of the Healthcare Cost and Utilization Project for the years 2002-2016. Neonates with HLHS were identified by ICD-9 and ICD-10 codes. Hospital and patient factors associated with inpatient mortality were analyzed. Overall, 18,867 neonates met the criteria of inclusion; a total of 3813 patients died during the hospitalization (20.2%). In-hospital mortality decreased over the years of the study (27.0% in 2002 vs. 18.3% in 2016). Extracorporeal membrane oxygenation utilization was 8.1%. Univariate and multivariate logistic regression analyses were used to identify risk factors for in-hospital mortality in infants with hypoplastic left heart syndrome. Independent non-modifiable risk factors for mortality were birth weight < 2500 g (Adjusted odds ratio (aOR) 2.16 [1.74-2.69]), gestational age < 37 weeks (aOR 1.73 [1.42-2.10]), chromosomal abnormalities (aOR 3.07 [2.60-3.64]) and renal anomalies (aOR 1.34 [1.10-1.61]). Independent modifiable risk factors for mortality were being transferred-in from another hospital (aOR 1.15 [1.03-1.29]), use of extracorporeal membrane oxygenation (aOR 12.74 [10.91-14.88]). Receiving care in a teaching hospital is a modifiable variable, and it decreased the odds of mortality (aOR 0. 78 [0.64-0.95]). In conclusion, chromosomal anomalies, Extra Corporeal Membrane Oxygenation, gestational age < 37 weeks or birth weight < 2500 g were associated with increased odds of mortality. Modifiable variables as receiving care at birth center and in a hospital designated as a teaching hospital decreased the odds of mortality.

Outcomes of truncus arteriosus repair and predictors of mortality.

OBJECTIVE: The objective of this study was to identify patient and hospitalization characteristics associated with in-hospital mortality in infants with truncus arteriosus. METHODS: We conducted a retrospective analysis of a large administrative database, the National Inpatient Sample data set of the Healthcare Cost and Utilization Project for the years 2002 to 2017. We also sought to evaluate the resource utilization in the subgroup of subjects with truncus arteriosus and 22q11.2 deletion syndrome. Neonates with truncus arteriosus were identified by ICD-9 and ICD-10 codes. Hospital and patient factors associated with inpatient mortality were analyzed. RESULTS: Overall, 3009 neonates met inclusion criteria; a total of 326 patients died during the hospitalization (10.8%). Extracorporeal membrane oxygenation utilization was 7.1%. Univariate and multivariate logistic regression analyses were used to identify risk factors for in-hospital mortality. Significant risk factors for mortality were prematurity (adjusted odds ratio [aOR] = 2.43; 95% confidence interval [CI]: 1.40-4.22; P = .002), diagnosis of stroke (aOR = 26.2; 95% CI: 10.1-68.1; P < .001), necrotizing enterocolitis (aOR = 3.10; 95% CI: 1.24-7.74; P = .015) and presence of venous thrombosis (aOR = 13.5; 95% CI: 6.7-27.2; P < .001). Patients who received extracorporeal membrane oxygenation support or had cardiac catheterization procedure during the hospitalization had increased odds of mortality (aOR = 82.0; 95% CI: 44.5-151.4; P < .001, and aOR = 1.65; 95% CI: 0.98-2.77; P = .060, respectively). CONCLUSION: 22q11.2 deletion syndrome was associated with an inverse risk of death despite having more noncardiac comorbidities; this patient subpopulation also had a higher length of stay and increased cost of hospitalization.

Neonatal outcomes of preterm infants with in-utero exposure to drugs of substance use: US national data.

BACKGROUND: Infants exposed prenatally to drugs of substance use are at increased risk for seizures, strabismus, feeding difficulty, and neurodevelopmental delays. Exposed preterm infants may have additional morbidities related to prematurity. There is limited literature on national outcomes of preterm infants exposed to drugs of substance use. We aimed to evaluate the trends and neonatal outcomes of preterm infants born in the USA who were exposed in-utero to drugs of substance use. METHODS: Retrospective cohort study of preterm live born (<37 weeks gestation) exposed in-utero to opioids, hallucinogens, or cocaine in the Healthcare Cost and Utilization Project database from 2002 to 2017. Neonatal outcomes were identified using international classification of diseases 9&10 codes. RESULTS: Of the 54,469,720 live-born infants, 7.7% (4,194,816) were preterm, and 58 679 (1.4%) were exposed in-utero to maternal opioids/hallucinogens (n = 39,335) or cocaine (n = 19,344). There was a trend for increased exposure to opioids/hallucinogens (Z score = 76.14, p < 0.001) during the study period. Exposed preterm infants had significantly more neurological anomalies, intra-ventricular hemorrhage and periventricular leukomalacia (p < 0.001). CONCLUSIONS: There was a trend for increased in-utero exposure to opioids and hallucinogens in the preterm infants in the USA. Exposed preterm infants had more neurological morbidities.

Primary anorectal melanoma mimicking polyp in a scleroderma patient: a case report.

Primary anorectal melanoma is an extremely rare malignancy of the gastrointestinal tract with a very poor prognosis. Due to the late presentation, most patients with primary anorectal melanoma are diagnosed at advanced stages. Scleroderma is an autoimmune disease characterized by fibrosis of the skin and visceral organs. There is an increased risk of developing cancer in scleroderma patients. Case Presentation: A 57-year-old Syrian female diagnosed with localized scleroderma complained of a sensation of a mass in her anal area. She was diagnosed with primary rectal melanoma and was put on neoadjuvant radiotherapy. Following the radiotherapy, the endoscopy revealed several black lesions in her anal canal, and thus abdominoperineal resection was conducted. Discussion and Conclusion: Malignant melanoma can occur in unsuspected locations such as the anal canal. Novel therapies like anti-CTLA4 drugs have proven efficient in controlling the disease. The lack of data in the literature on this malignancy and the absence of guidelines make it challenging for an optimal approach.

Age-related macular degeneration and genetic polymorphisms of glutathione S-transferases M1 (GSTM1) and T1 (GSTT1).

The aim of this study is to understand the multifactorial causes of age-related macular degeneration (ARMD), and, therefore, it is reasonable to investigate whether genetic polymorphisms of antioxidant enzymes (GSTM1 and GSTT1) contribute to the development of ARMD. This study consisted of 112 subjects (44 females, 68 males) with exudative ARMD, who were recruited from Khalili Hospital ophthalmic clinic in Shiraz (southern Iran), referred by vitreoretinal surgeon. Also 112 sex-matched controls (44 females, 68 males) were randomly selected from unrelated volunteers in the same clinic. We excluded patients and controls with cataract or past history of cataract surgery, asthma, past history of malignancy, cardiovascular disease that on medication and known cases of glaucoma, because these traits were associated with GSTM1 and/or GSTT1 polymorphisms. There was no association between polymorphisms of neither GSTM1 nor GSTT1 and risk of ARMD. The combination genotypes of GSTM1 and GSTT1 were not associated with the risk of ARMD. We considered the time of deterioration of vision as the time of onset of exudative ARMD. The Kaplan-Meier analysis revealed that there was significant difference between genotypes of GSTM1 (log rank statistic = 7.03, df = 1, P = 0.008). The age at onset among GSTM1 null genotype was lower than the active genotype of GSTM1. Our results support the hypothesis that the protein encoded by the GSTM1 gene might have a protective function against oxidative stress in retina. Since the age at onset is influenced by the GSTM1 polymorphism, this implies that GSTM1 is a modifier gene.

Outcomes of complete surgical repair versus palliative intervention in neonates with Tetralogy of Fallot.

Using the US National Inpatient Sample dataset (2010 to 2018), we compared outcomes of neonates with Tetralogy of Fallot who had early primary surgical repair (1726 neonate) and those who had staged palliative intervention with transcatheter (1702 neonate) or surgical palliative shunt (2661 neonate).

Epidemiologic evaluation of inhaled nitric oxide use among neonates with gestational age less than 35 weeks.

BACKGROUND AND OBJECTIVES: The use of inhaled nitric oxide (iNO) in +late preterm and term infants with pulmonary hypertension is Food and Drug Administration (FDA) approved and has improved outcomes and survival. iNO use is not FDA approved for preterm infants and previous studies show no mortality benefit. The objectives were 1) to determine the usage of iNO among preterm neonates <35 weeks before and after the 2010 National Institutes of Health consensus statement and 2) to evaluate characteristics and outcomes among preterm neonates who received iNO. METHODS: This is a population-based cross-sectional study. Billing and procedure codes were used to determine iNO usage. Data were queried from the National Inpatient Sample from 2004 to 2016. Neonates were included if gestational age was <35 weeks. The epochs were spilt into 2004-2010 (Epoch 1) and 2011-2016 (Epoch 2). Prevalence of iNO use, mortality, bronchopulmonary dysplasia (BPD), intraventricular hemorrhage, length of stay, mechanical ventilation, and cost of hospitalization. RESULTS: There were 4865 preterm neonates <35 weeks who received iNO. There was a significant increase in iNO use during Epoch 2 (p < 0.001). There was significantly higher use in Epoch 2 among neonates small for gestational age (SGA) 2.3% versus 7.2%, congenital heart disease (CHD) 11.1% versus 18.6%, and BPD 35.2% versus 46.8%. Mortality was significantly lower in Epoch 2 19.8% versus 22.7%. CONCLUSION: Usage of iNO was higher after the release of the consensus statement. The increased use of iNO among preterm neonates may be targeted at specific high-risk populations such as SGA and CHD neonates. There was lower mortality in Epoch 2; however, the cost was doubled.

Neonatal Ebstein anomaly national outcomes from 2000 to 2018 using the National Inpatient Sample.

BACKGROUND: The prevalence, morbidity, and mortality associated with Ebstein anomaly (EA) remains poorly characterized in neonates. EA is a rare form of congenital heart disease (CHD) with significant heterogeneity. OBJECTIVE: To determine the recent, 2000-2018, prevalence, mortality, outcomes, and healthcare utilization of infants admitted at ≤28 days of life with EA in comparison to other critical congenital heart defects (CCHD) in the United States using a national data set. METHODS: The National Inpatient Sample (NIS) from the Healthcare Cost and Utilization Project (HCUP) was queried for infants admitted for any reason at ≤28 days of life with a diagnosis of EA between 2000 and 2018 using ICD-9 and 10 codes in the United States. Patient characteristics, morbidity, mortality, and healthcare utilization were evaluated for EA and compared with other CCHD. RESULTS: From 2000 to 2018 a total of 68,312,952 neonatal admissions were identified, of them 4,398 neonates with isolated EA were identified, representing 7 per 100,000 neonatal admissions and 2.2% of CCHD admissions (4,398/197,881). The number of new EA cases ranged from 138 to 375 per year. In-hospital mortality was 12.3% and surgical repair occurred in 4.2% for infants with EA. There were 470 deaths without surgical repair which is 86.6% of the mortality. Arrhythmias were diagnosed in 10.6% and ECMO was used for 2.6% of neonates with EA. CONCLUSION: EA is a rare form of CHD. The prevalence has remained stable over the 19 years whereas other congenital heart defects have had an increase. The mortality in neonates with EA was significantly higher than in pooled CCHD; the burden of mortality occurred in the neonates without surgical intervention.