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Assessment Of Diastolic Function In Single-Ventricle Patients After The Fontan Procedure., Renee Margossian, Lynn A. Sleeper, Gail D. Pearson, Piers C. Barker, Luc Mertens, Michael D. Quartermain, Jason T. Su, Girish S. Shirali, Shan Chen, Steven D. Colan, Pediatric Heart Network Investigators

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Patients with functional single ventricles after the Fontan procedure have abnormal cardiac mechanics. The aims of this study were to determine factors that influence diastolic function and to describe associations of diastolic function with current clinical status.

METHODS: Echocardiograms were obtained as part of the Pediatric Heart Network Fontan Cross-Sectional Study. Diastolic function grade (DFG) was assessed as normal (grade 0), impaired relaxation (grade 1), pseudonymization (grade 2), or restrictive (grade 3). Studies were also classified dichotomously (restrictive pattern present or absent). Relationships between DFG and pre-Fontan variables (e.g., ventricular morphology, age at Fontan, history of volume-unloading surgery) and …

Functional Health Status In Children And Adolescents After Fontan: Comparison Of Generic And Disease-Specific Assessments., Brian W. Mccrindle, Victor Zak, Victoria L. Pemberton, Linda M. Lambert, Victoria L. Vetter, Wyman W. Lai, Karen Uzark, Renee Margossian, Andrew M. Atz, Amanda Cook, Jane W. Newburger, Pediatric Heart Network Investigators, Girish S. Shirali

Manuscripts, Articles, Book Chapters and Other Papers

PURPOSE: The aim of this study was to compare associations between generic versus disease-specific functional health status assessments and patient and clinical characteristics for patients with severe congenital heart disease.

METHODS: This was a cross-sectional observational study involving 325 single ventricle patients, aged 10-18 years, after Fontan procedure. Enrolled patients underwent a medical history review, laboratory testing, and assessment of the functional health status by completion of the generic Child Report Child Health Questionnaire and the disease-specific Congenital Heart Adolescent and Teenage questionnaire. Correlated conceptually equivalent domains from both questionnaires were identified and their associations with patient and clinical variables …

Factors Impacting Echocardiographic Imaging After The Fontan Procedure: A Report From The Pediatric Heart Network Fontan Cross-Sectional Study., Richard V. Williams, Renee Margossian, Minmin Lu, Andrew M. Atz, Timothy J. Bradley, Michael Jay Campbell, Steven D. Colan, Dianne Gallagher, Wyman W. Lai, Gail D. Pearson, Ashwin Prakash, Girish S. Shirali, Meryl S. Cohen, Pediatric Heart Network Investigators

Manuscripts, Articles, Book Chapters and Other Papers

Echocardiographic image quality in Fontan survivors may be limited by a variety of factors. We sought to describe echocardiographic quality and factors associated with study quality in subjects participating in the Pediatric Heart Network Fontan Cross-Sectional Study. Echocardiograms were obtained at 7 clinical sites using a standard protocol. Quality grading and analysis were performed by a core laboratory. Univariate and multivariable modeling were performed to assess factors associated with quality and ability to obtain images sufficient for prespecified quantitative analysis. A total of 543 echocardiograms were obtained. The quality of echocardiograms improved over the duration of the study. The great …

Is Laparoscopy A Risk Factor For Bile Duct Injury During Cholecystectomy?, Terrence M. Fullum, Stephanie R. Downing, Gezzer Ortega, David C. Chang, Tolulope A. Oyetunji, Kendra Van Kirk, Daniel D. Tran, Ian Woods, Edward E. Cornwell, Patricia L. Turner

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND AND OBJECTIVES: Previously, risk factors for bile duct injury have been identified as acute cholecystitis, male gender, older age, aberrant biliary anatomy, and laparoscopic cholecystectomy.

METHODS: A retrospective analysis of the Nationwide Inpatient Sample from 1998 to 2006 was performed with an inclusion criterion of cholecystectomy performed on hospital day 0 or 1. Patient- and hospital-level factors potentially associated with bile duct injury were examined by logistic regression.

RESULTS: A total of 377,424 cholecystectomy patients were identified. There were 1124 bile duct injuries (0.30%), with 177 (0.06%) in the laparoscopic cholecystectomy group and 947 (1.46%) in the open cholecystectomy …

State-By-State Variation In Emergency Versus Elective Colon Resections: Room For Improvement., Augustine C. Obirieze, Mehreen Kisat, Caitlin W. Hicks, Tolulope A. Oyetunji, Eric B. Schneider, Darrell J. Gaskin, Elliott R. Haut, David T. Efron, Edward E. Cornwell, Adil H. Haider

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Compared with elective surgical procedures, emergency procedures are associated with higher cost, morbidity, and mortality. This study seeks to investigate potential state-by-state variations in the incidence of emergent versus elective colon resections.

METHODS: A retrospective analysis of all adult patients (aged ≥18 years) included in the Nationwide Inpatient Sample from 2005 to 2009 who underwent hemicolectomy (right or left) or sigmoidectomy was conducted. Discharge-level weights were applied, and generalized linear models were used to assess the odds of a patient undergoing emergent versus elective colon surgery nationally and for each state after adjusting for patient and hospital factors. Odds …

Open Versus Laparoscopic Hiatal Hernia Repair., Terrence M. Fullum, Tolulope A. Oyetunji, Gezzer Ortega, Daniel D. Tran, Ian M. Woods, Olusola Obayomi-Davies, Orighomisan Pessu, Stephanie R. Downing, Edward E. Cornwell

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: The literature reports the efficacy of the laparoscopic approach to paraesophageal hiatal hernia repair. However, its adoption as the preferred surgical approach and the risks associated with paraesophageal hiatal hernia repair have not been reviewed in a large database.

METHOD: The Nationwide Inpatient Sample dataset was queried from 1998 to 2005 for patients who underwent repair of a complicated (the entire stomach moves into the chest cavity) versus uncomplicated (only the upper part of the stomach protrudes into the chest) paraesophageal hiatal hernia via the laparoscopic, open abdominal, or open thoracic approach. A multivariate analysis was performed controlling for …

Surgical Management Of Complete Atrioventricular Septal Defect: Associations With Surgical Technique, Age, And Trisomy 21., Andrew M. Atz, John A. Hawkins, Minmin Lu, Meryl S. Cohen, Steven D. Colan, James Jaggers, Ronald V. Lacro, Brian W. Mccrindle, Renee Margossian, Ralph S. Mosca, Lynn A. Sleeper, L Luann Minich, Pediatric Heart Network Investigators, Girish S. Shirali

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVES: We sought to evaluate the contemporary results after repair of a complete atrioventricular septal defect and to determine the factors associated with suboptimal outcomes.

METHODS: The demographic, procedural, and outcome data were obtained within 1 and 6 months after repair of a complete atrioventricular septal defect in 120 children in a multicenter observational study from June 2004 to 2006.

RESULTS: The median age at surgery was 3.7 months (range, 9 days to 1.1 years). The type of surgical repair was a single patch (18%), double patch (72%), and a single atrial septal defect patch with primary ventricular septal defect …

Relation Of Size Of Secondary Ventricles To Exercise Performance In Children After Fontan Operation., Ashwin Prakash, Thomas G. Travison, Mark A. Fogel, Lynne M. Hurwitz, Andrew J. Powell, Beth F. Printz, Michael D. Puchalski, Girish S. Shirali, Shi-Joon Yoo, Tal Geva, Pediatric Heart Network Investigators

Manuscripts, Articles, Book Chapters and Other Papers

The effects of the nondominant or secondary ventricle on the Fontan circulation are not known. The present study used cardiac magnetic resonance imaging to investigate the relations between secondary ventricular size and global cardiac performance. The Fontan cross-sectional study collected data from 7 centers participating in the Pediatric Heart Network. Subjects with complete cardiac magnetic resonance imaging data and an identifiable secondary ventricle were included in the analysis. Relationships between body surface area-adjusted parameters of the secondary ventricle (mass, end-diastolic volume, mass/volume ratio, and stroke volume) and the following measures were assessed. These measures included the percentage of predicted peak …

Asian Race/Ethnicity As A Risk Factor For Bile Duct Injury During Cholecystectomy., Stephanie R. Downing, Ghazala Datoo, Tolulope A. Oyetunji, Terrence Fullum, David C. Chang, Nita Ahuja

Manuscripts, Articles, Book Chapters and Other Papers

Iatrogenic bile duct injury (BDI) is an uncommon but serious complication of cholecystectomy, with identified risk factors of acute cholecystitis, male sex, older age, and aberrant biliary anatomy. The Nationwide Inpatient Sample (1998-2006) was queried for cholecystectomy performed on hospital day 0 or 1. Bile duct injury repair procedure codes were used as a surrogate for BDI. We identified 377,424 patients who underwent cholecystectomy, with 1124 BDIs (0.3%). On multivariate logistic regression analysis, Asian race/ethnicity was a significant risk factor for BDI (odds ratio [OR], 2.26; 95% confidence interval [CI], 1.59-3.23; P < .001). This persisted for laparoscopic (OR, 2.62; 95% CI, 1.28-5.39; P = .009) and open (2.21; 1.59-3.07; P < .001) cholecystectomies. No other race/ethnicity was identified as a risk factor for BDI. We report a new finding that Asian race/ethnicity is a significant risk factor for BDI in laparoscopic and open cholecystectomies.

Parent- Versus Child-Reported Functional Health Status After The Fontan Procedure., Linda M. Lambert, L Luann Minich, Jane W. Newburger, Minmin Lu, Victoria L. Pemberton, Ellen A. Mcgrath, Andrew M. Atz, Mingfen Xu, Elizabeth Radojewski, Darlene Servedio, Brian W. Mccrindle, Pediatric Heart Network Investigators, Girish S. Shirali

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVE: We sought to compare perceptions of functional health status between children who had undergone a Fontan procedure and their parents.

METHODS: Fontan procedure survivors 10 to 18 years of age were included in the study if the child completed the Child Health Questionnaire (CHQ) and the parent completed the parent form to assess the child's functional health status. Comparisons were made between raw domain scores for the parent- and child-completed CHQs.

RESULTS: Between March 2003 and April 2004, 1078 Fontan survivors were screened. Of the 546 eligible and consented patients, 354 were 10-18 years of age and 328 parent/child …

Functional State Following The Fontan Procedure., Ismee A. Williams, Lynn A. Sleeper, Steven D. Colan, Minmin Lu, Elizabeth A. Stephenson, Jane W. Newburger, Welton M. Gersony, Meryl S. Cohen, James F. Cnota, Andrew M. Atz, Richard V. Williams, Renee Margossian, Andrew J. Powell, Mario P. Stylianou, Daphne T. Hsu, Pediatric Heart Network Investigators, Girish S. Shirali

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Despite improvements in outcomes after completion of the Fontan circulation, long-term functional state varies. We sought to identify pre- and postoperative characteristics associated with overall function.

METHODS AND RESULTS: We analyzed data from 476 survivors with the Fontan circulation enrolled in the Pediatric Heart Network Fontan Cross-sectional Study. Mean age at creation of the Fontan circulation was 3.4 plus or minus 2.1 years, with a range from 0.7 to 17.5 years, and time since completion was 8.7 plus or minus 3.4 years, the range being from 1.1 to 17.3 years. We calculated a functional score for the survivors by …

Laparoscopic Treatment Of Pancreatic Pseudocysts In Children., Suzanne M. Yoder, Steven Rothenberg, Kuojen Tsao, Mark L. Wulkan, Todd A. Ponsky, Shawn D. St Peter, Daniel J. Ostlie, Timothy D. Kane

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Pancreatic pseudocysts are problematic sequelae of pancreatitis or pancreatic trauma causing persistent abdominal pain, nausea, and gastric outlet obstruction. Due to the low volume of disease in children, there is scant information in the literature on the operative management of pseudocysts with minimally invasive techniques. We conducted a multi-institutional review to illustrate several technical variations utilized in achieving laparoscopic cystgastrostomy in the pediatric population.

METHODS: A retrospective review was conducted of all patients who underwent laparoscopic cystgastrostomy in five institutions. Patient data, operative techniques, and postoperative course were analyzed.

RESULTS: There were 13 patients with a mean age of …

Design And Rationale Of A Randomized Trial Comparing The Blalock-Taussig And Right Ventricle-Pulmonary Artery Shunts In The Norwood Procedure., Richard G. Ohye, J William Gaynor, Nancy S. Ghanayem, Caren S. Goldberg, Peter C. Laussen, Peter C. Frommelt, Jane W. Newburger, Gail D. Pearson, Sarah Tabbutt, Gil Wernovsky, Lisa M. Wruck, Andrew M. Atz, Steve D. Colan, James Jaggers, Brian W. Mccrindle, Ashwin Prakash, Michael D. Puchalski, Lynn A. Sleeper, Mario P. Stylianou, Lynn Mahony, Pediatric Heart Network Investigators, Girish S. Shirali

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVE: The initial palliative procedure for patients born with hypoplastic left heart syndrome and related single right ventricle anomalies, the Norwood procedure, remains among the highest risk procedures in congenital heart surgery. The classic Norwood procedure provides pulmonary blood flow with a modified Blalock-Taussig shunt. Improved outcomes have been reported in a few small, nonrandomized studies of a modification of the Norwood procedure that uses a right ventricle-pulmonary artery shunt to provide pulmonary blood flow. Other nonrandomized studies have shown no differences between the two techniques.

METHODS: The Pediatric Heart Network designed a randomized clinical trial to compare outcomes for …

Management Of Pediatric Acute Appendicitis In The Computed Tomographic Era., Kuojen Tsao, Shawn D. St Peter, Patricia A. Valusek, Troy L. Spilde, Scott J. Keckler, Abhilash Nair, Daniel J. Ostlie, G W. Holcomb Iii

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND/PURPOSE: The treatment options for complicated appendicitis in children continue to evolve. Optimal management of complicated appendicitis relies on an accurate preoperative diagnosis. We examined the accuracy of our preoperative diagnosis including computed tomography (CT) and the influence on the management of children with perforated and nonperforated appendicitis.

METHODS: Following IRB approval, a 6-year review of all patients that underwent an appendectomy for suspected appendicitis was performed. Treatments included immediate operations and initial nonoperative management (antibiotic therapy +/- percutaneous drainage of abscess). Appendicitis was confirmed by histological examination.

RESULTS: One thousand seventy-eight patients underwent appendectomy for suspected appendicitis. Preoperative CT …

Current Application Of Thoracoscopy In Children., Kuojen Tsao, Shawn D. St Peter, Susan W. Sharp, Abhilash Nair, Walter S. Andrews, Ronald J. Sharp, Charles L. Snyder, Daniel J. Ostlie, G W. Holcomb Iii

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: The safety and efficacy of thoracoscopy for thoracic lesions and conditions in children is evolving. Our experience with thoracoscopy has expanded in recent years. Therefore, we reviewed our most recent 7-year experience to examine the current applications for thoracoscopy in children.

METHODS: A retrospective review of all patients undergoing a thoracoscopic operation at Children's Mercy Hospital (Kansas City, MO) between January 1, 2000, and June 18, 2007, was performed. Data points reviewed included patient demographics, type of operation, final diagnosis, complications, and recovery.

RESULTS: During the study period, 230 children underwent 231 thoracoscopic procedures. The mean age was 9.6 …

Dobutamine Stress Echocardiography For Assessing Coronary Artery Disease After Transplantation In Children., R L. Larsen, P M. Applegate, D A. Dyar, P A. Ribeiro, S D. Fritzsche, N F. Mulla, Girish S. Shirali, M A. Kuhn, R E. Chinnock, P M. Shah

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVES: The purpose of this study was to determine the feasibility, safety and diagnostic accuracy of dobutamine stress echocardiography (DSE) for evaluating posttransplant coronary artery disease (TxCAD) in children, and to determine the frequency of selected cardiac events after normal or abnormal DSE.

BACKGROUND: Posttransplant coronary artery disease is the most common cause of graft loss (late death or retransplantation) after cardiac transplantation (CTx) in children. Coronary angiography, routinely performed to screen for TxCAD, is an invasive procedure with limited sensitivity. The efficacy of DSE for detecting atherosclerotic coronary artery disease is established, but is unknown in children after CTx. …

Posttransplant Recoarctation Of The Aorta: A Twelve Year Experience., Girish S. Shirali, C E. Cephus, M A. Kuhn, K K. Ogata, L K. Vander Dussen, R E. Chinnock, N F. Mulla, J K. Johnston, L L. Bailey, S R. Gundry, A J. Razzouk, R L. Larsen

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVES: This study was undertaken to investigate the incidence of posttransplant recoarctation of the aorta, delineate the mode of presentation, identify risk factors that predict recoarctation and examine the results of intervention for posttransplant recoarctation.

BACKGROUND: Patients with aortic arch hypoplasia require extended arch reconstruction at transplant, with an inherent possibility of subsequent recoarctation of the aorta.

METHODS: This was a retrospective review of all children (ageyears) who underwent cardiac transplantation over a 10-year period. Collected data included pretransplant diagnosis, details of the transplant procedure and posttransplant data including development of recoarctation of the aorta, interventions for recoarctation and the …