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Uppu S.C.,Divisions of Pediatric Cardiology | Gossett J.M.,University of Arkansas at Little Rock | Yan K.,Stanford University | Dao D.T.,Stanford University | And 3 more authors.
ASAIO Journal | Year: 2013

Our objective was to evaluate morbidity and mortality associated with extracorporeal membrane oxygenation (ECMO) in children with genetic syndromes and heart disease. We conducted a retrospective review of all children with heart disease and genetic syndromes receiving ECMO during the period January 2000 and March 2012 at Arkansas Children's Hospital, Little Rock. The medical charts were reviewed to obtain the following variables: demographic information, medical and surgical history, laboratory and microbiological, information on organ dysfunction, and outcome characteristics. The outcome variables evaluated in this report included: hospital length of stay (LOS), survival to hospital discharge, and current survival. Outcome data were compared among critically ill children with and without syndromes. During the study period, there were 377 ECMO runs in 336 children with heart disease. Of these, 43 ECMO runs occurred in children with genetic syndromes whereas 334 ECMO runs occurred in children with no genetic abnormality. Children in the group with underlying genetic syndrome were older at the time of ECMO cannulation than the group with no syndrome. During the ECMO run, hospital LOS and mortality were similar in children with and without underlying genetic abnormality. Among genetically abnormal patients, renal insufficiency and need for dialysis were associated with mortality. In this group, 24 patients (56%) were discharged alive. However, only 10 patients are living to date in this cohort. ECMO can be used in children with heart disease and genetic syndromes with good results. The survival rate is high and the complication rate is low. Copyright © 2013 by the American Society for Artificial Internal.


Rusconi P.G.,Divisions of Pediatric Cardiology | Rusconi P.G.,University of Miami | Ratnasamy C.,Divisions of Pediatric Cardiology | Ratnasamy C.,University of Miami | And 6 more authors.
American Heart Journal | Year: 2010

Background: Increasing serum levels of N-terminal pro-hormone brain natriuretic peptide (NT-proBNP) are associated with worsening heart failure (HF) in adults. We determined whether changes in NT-proBNP level are associated with changes in symptoms and left ventricular (LV) systolic function and remodeling in children with HF secondary to dilated cardiomyopathy. Methods: We retrospectively examined associations between serum NT-proBNP levels and NYHA/Ross functional class, LV systolic and diastolic diameter (LVSD-z and LVDD-z), LV ejection fraction (LVEF), and LV shortening fraction (LVSF-z) using generalized linear mixed models. Fluctuation in functional class of subjects was also modeled using logistic regression and receiver operating characteristic (ROC) curves. Results: In 36 children (14 males), a 10-fold increase in NT-proBNP serum levels was associated (P < .001) with a 9.8% decrease in LVEF, a 3.25-unit drop in LVSF-z, a 1.53-unit increase in LVDD-z, a 2.64-unit increase in LVSD-z, and an increased odds of being in functional class III/IV (OR 85.5; 95% CI, 10.9 to 671.0). An NT-proBNP level greater than 1000 pg/mL identified children constantly or intermittently in functional class III-IV with 95% sensitivity and 80% specificity. The reliability of a single NT-proBNP value was 0.61, but the means for two and three NT-proBNP values were 0.76 and 0.82, respectively. Conclusions: In children with HF, NT-proBNP is associated with cardiac symptoms and indices of LV systolic dysfunction and remodeling. NT-proBNP >1000 pg/mL identifies highly symptomatic children. Within subject serial measurements of NT-proBNP are needed for a reliable and accurate determination of disease status and/or course. © 2010 Mosby, Inc.


Oztarhan K.,Divisions of Pediatric Cardiology | Gedikbasi A.,Istanbul Bakirkoy Maternity and Children Diseases Hospital | Yildirim D.,Istanbul Bakirkoy Maternity and Children Diseases Hospital | Arslan O.,Istanbul Bakirkoy Maternity and Children Diseases Hospital | And 4 more authors.
Congenital Anomalies | Year: 2010

The aim of this study was to determine the distribution of cases associated with congenital abnormalities during the following three periods: pregnancy, birth, and the neonatal period. This was a retrospective study of cases between 2002 and 2006. All abnormal pregnancies, elective terminations of pregnancies, stillbirths, and births with congenital abnormalities managed in the Neonatology Unit were classified based on the above distribution scheme. During the 5-year study period, 1906 cases with congenital abnormalities were recruited, as follows: 640 prenatally detected and terminated cases, with most abnormalities related to the central nervous system, chromosomes, and urogenital system (56.7%, 12.7%, and 8.9%, respectively); 712 neonates with congenital abnormalities (congenital heart disease [49.2%], central nervous system abnormalities [14.7%], and urogenital system abnormalities [12.9%]); and hospital stillbirths, of which 34.2% had malformations (220 prenatal cases [34.4%] had multiple abnormalities, whereas 188 liveborn cases [26.4%] had multiple abnormalities). The congenital abnormalities rate between 2002 and 2006 was 2.07%. Systematic screening for fetal anomalies is the primary means for identification of affected pregnancies. © 2010 The Authors. Congenital Anomalies © 2010 Japanese Teratology Society.


Ruccione K.S.,LIFE Cancer Survivorship and Transition Program | Ruccione K.S.,Childrens Hospital Los Angeles | Ruccione K.S.,University of Southern California | Wood J.C.,Divisions of Pediatric Cardiology | And 8 more authors.
Cancer Epidemiology Biomarkers and Prevention | Year: 2014

Background: Childhood cancer survivors (CCS) receiving packed red blood cell (PRBC) transfusions may have increased risk for vital organ iron deposition causing serious late effects.Methods: This cross-sectional cohort study of a CCS cohort quantified organ iron content by magnetic resonance imaging. Iron status by serum markers and hemochromatosis gene mutation status were assessed.Results: Seventy-five patients who had received a range (0-392 mL/kg) of cumulative PRBC transfusion volumes were enrolled (median age 14 years, range 8-25.6 years at evaluation). Median follow-up time was 4.4 years, and median time since last transfusion was 4.9 years. Cancer diagnoses included acute lymphoblastic or myelogenous leukemia (ALL/AML; n=33) and solid tumors (n=42). Liver and pancreatic iron concentrations were elevated in 36 of 73 (49.3%) and 19 of 72 (26.4%) subjects, respectively. Cardiac iron concentration was not increased in this cohort. In multivariate analysis, cumulative PRBC volume (P < 0.0001) and older age at diagnosis (P < 0.0001) predicted elevated liver iron concentration.Impact: These findings have implications for development of monitoring and management guidelines for cancer patients and survivors at risk for IO, exploration of the additive risk of liver/pancreatic damage from chemotherapeutic exposures, and health education to minimize further liver/pancreatic damage from exposures such as excessive alcohol intake and hepatotoxic medications.Conclusions: Iron overload (IO) may occur in children and adolescents/young adults treated for cancer and is associated with cumulative PRBC transfusion volume and age at diagnosis. © 2014 AACR.


Koestenberger M.,Divisions of Pediatric Cardiology | Koestenberger M.,Medical University of Graz | Nagel B.,Divisions of Pediatric Cardiology | Ravekes W.,Johns Hopkins University | And 7 more authors.
Neonatology | Year: 2011

Background: The tricuspid annular plane systolic excursion (TAPSE) is an echocardiographic measurement to assess right ventricular systolic function in adults and children. Objective: We determined growth- and birth weight-related changes of TAPSE to establish reference values in preterm and term neonates. Methods: A prospective study was conducted in a group of 258 preterm and term neonates (age: 25+0 to 40+6 weeks of gestation, birth weight: 530-4,200 g). Results: The TAPSE ranged from a mean of 0.44 cm (Z-score ±2: 0.30-0.59 cm) in preterm neonates in the 26th week of gestation to 1.03 cm (Z-score ±2: 0.85-1.21 cm) in term neonates in the 41st week of gestation. The TAPSE values increased in a linear way from the 26th to 41st week of gestation. TAPSE, week of gestation and weight are strongly correlated: Pearson's correlation coefficient was 0.93 for week of gestation - TAPSE (p < 0.001), 0.93 for week of gestation - birth weight (p < 0.001), and 0.89 for birth weight - TAPSE (p < 0.001). There was no statistically significant difference of normal TAPSE values between female and male patients (p = 0.987). Conclusion: Z-scores of TAPSE values were calculated and percentile charts were established to serve as reference data for ready application in preterm and term neonates with structurally normal hearts and with congenital heart disease in the future. Copyright © 2011 S. Karger AG, Basel.

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