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Amy H Schultz, Richard F Ittenbach, Marsha Gerdes, Gail P Jarvik, Gil Wernovsky, Judy Bernbaum, Cynthia Solot, Robert R Clancy, Susan C Nicolson, Thomas L Spray, Donna McDonald-McGinn, Elaine Zackai, J William Gaynor
OBJECTIVES: We sought to assess the effect of congenital heart disease requiring infant surgery with cardiopulmonary bypass on neurodevelopmental outcomes and growth at 4 years of age, while matching for gestational age, socioeconomic status, maternal gestational conditions, home environment, and parental intelligence by studying multiple-gestation births. METHODS: We performed within-family comparison of 14 multiple-gestation births in which 1 child had congenital heart disease requiring surgery with cardiopulmonary bypass at ≤6 months of age...
July 2017: Journal of Thoracic and Cardiovascular Surgery
Megan L Ringle, Gil Wernovsky
Throughout the past few decades, advances in cardiology, neonatal intensive care, and surgical techniques have resulted in a growing cohort of thriving school-aged children with previously lethal complex congenital heart diseases. While survival has increased, there remains significant morbidity following repair including neurodevelopmental sequelae. Compared to children with a structurally normal heart, these infants and children have a higher frequency of abnormalities in tone, feeding, and delayed developmental milestones, as well as challenges with speech and learning disabilities, while a higher proportion of adolescents suffer from problems with processing speed, executive function, and a unique set of medical hardships related to exercise intolerance and obesity, medication burden, and mental health comorbidities...
December 2016: Seminars in Perinatology
Gil Wernovsky
OBJECTIVES: The objectives of this review are to discuss the physiology, perioperative management, surgical correction, and outcomes of infants with transposition of the great arteries and common variants undergoing the arterial switch operation. DATA SOURCE: MEDLINE and PubMed. CONCLUSION: The widespread adoption of the arterial switch operation for transposition of great arteries has been one of the more gratifying advances in pediatric cardiovascular care, and represents the simultaneous improvements in diagnostics, surgical and bypass techniques, anesthesia in the neonate, improvements in intensive care technology, nursing strategies, and system-wide care delivery...
August 2016: Pediatric Critical Care Medicine
Gil Wernovsky, Daniel J Licht
OBJECTIVES: The objectives of this review are to discuss the scope of neurologic injuries in newborns with congenital heart disease, the mechanisms of injury, including prenatal, pre-, intra-, and postoperative factors, neurodevelopmental outcomes, and therapeutic strategies for the timely intervention and prevention of neurologic injury. DATA SOURCE: MEDLINE and PubMed. CONCLUSION: At the current time, important research is underway to 1) better understand the developing brain in the fetus with complex congenital heart disease, 2) to identify modifiable risk factors in the operating room and ICU to maximize long-term neurodevelopmental outcomes, and 3) develop strategies to improve family psychosocial health, childhood development, and health-related quality of life following hospital discharge...
August 2016: Pediatric Critical Care Medicine
Amy M O'Connor, Jo Wray, Ryan S Tomlinson, Amy Cassedy, Jeffrey P Jacobs, Kathy J Jenkins, Kate L Brown, Rodney C G Franklin, Lynn Mahony, Kathleen Mussatto, Jane W Newburger, Gil Wernovsky, Richard F Ittenbach, Dennis Drotar, Bradley S Marino
BACKGROUND: Surgical complexity and related morbidities may affect long-term patient quality of life (QOL). Aristotle Basic Complexity (ABC) score and Risk Adjustment in Congenital Heart Surgery (RACHS-1) category stratify the complexity of pediatric cardiac operations. The purpose of this study was to examine the relationship between surgical complexity and QOL and to investigate other demographic and clinical variables that might explain variation in QOL in pediatric cardiac surgical survivors...
2016: Journal of the American Heart Association
Gil Wernovsky, Stacey L Lihn, Melissa M Olen
Over the past 20 years, the successes of neonatal and infant surgery have resulted in dramatically changed demographics in ambulatory cardiology. These school-aged children and young adults have complex and, in some cases, previously unexpected cardiac and non-cardiac consequences of their surgical and/or transcatheter procedures. There is a growing need for additional cardiac and non-cardiac subspecialists, and coordination of care may be quite challenging. In contrast to hospital-based care, where inpatient care protocols are common, and perioperative expectations are more or less predictable for most children, ambulatory cardiologists have evolved strategies of care more or less independently, based on their education, training, experience, and individual styles, resulting in highly variable follow-up strategies...
May 2017: Cardiology in the Young
Elizabeth C Caris, Nicole Dempster, Gil Wernovsky, Catherine Butz, Trent Neely, Robin Allen, Jamie Stewart, Holly Miller-Tate, Rachel Fonseca, Karen Texter, Lisa Nicholson, Clifford L Cua
OBJECTIVES: Caring for children with congenital heart disease places significant stress on caregivers. Minimal data exist evaluating stress levels in caregivers of children with hypoplastic left heart syndrome (HLHS). The goal of this study was to obtain baseline stress scores for caregivers of children with HLHS and determine if associations exist between scores and specific caregiver factors. STUDY DESIGN: A cross-sectional study using a web-based survey targeted towards caregivers of children with HLHS was performed...
December 2016: Congenital Heart Disease
Punkaj Gupta, Michael J Robertson, Mallikarjuna Rettiganti, Paul M Seib, Gil Wernovsky, Barry P Markovitz, Janet Simsic, Joseph D Tobias
Little is known about the relationship of timing of extracorporeal membrane oxygenation (ECMO) initiation on patient outcomes after pediatric heart surgery. We hypothesized that increasing timing of ECMO initiation after heart surgery will be associated with worsening study outcomes. Patients aged ≤18 years receiving ECMO after pediatric cardiac surgery at a Pediatric Health Information System-participating hospital (2004-2013) were included. Outcomes evaluated included in-hospital mortality, composite poor outcome, prolonged length of ECMO, prolonged length of mechanical ventilation, prolonged length of ICU stay, and prolonged length of hospital stay...
June 2016: Pediatric Cardiology
Michael P Fundora, Enrique Oliver Aregullin, Gil Wernovsky, Elizabeth M Welch, Juan-Carlos Muniz, Nao Sasaki, Robert L Hannan, Redmond P Burke, Leo Lopez
OBJECTIVE: Determine the accuracy of echocardiography to diagnose coronary anatomy in transposition of the great arteries and to evaluate the effect of accuracy on surgical outcomes and changes in accuracy over time. DESIGN: Retrospective chart review of neonates admitted February 1999 to March 2013 with transposition. Coronary pattern from the preoperative echocardiogram and operative reports were collected and compared with determine diagnostic accuracy. Coronary patterns were further confirmed by intraoperative images taken during surgery...
December 2016: Congenital Heart Disease
Jun Sasaki, John C Dykes, Lisa J Sosa, Jane L Salvaggio, Milagros D Tablante, Jorge Ojito, Danyal M Khan, Robert L Hannan, Anthony F Rossi, Redmond P Burke, Gil Wernovsky
OBJECTIVES: Children with functional single ventricle undergoing the Fontan operation consume considerable resources. The purpose of this study is to evaluate pre- and intraoperative risk factors for longer hospital stay and to describe the perioperative course at a single institution over a 15-year period. DESIGN: Retrospective cohort study. SETTING: A single pediatric cardiac ICU. PATIENTS: All consecutive patients undergoing a first-time Fontan operation from 2000 to 2014...
May 2016: Pediatric Critical Care Medicine
Jeffrey P Jacobs, Gil Wernovsky, David S Cooper, Tom R Karl
In the domain of paediatric and congenital cardiac care, the stakes are huge. Likewise, the care of these children assembles a group of "A+ personality" individuals from the domains of cardiac surgery, cardiology, anaesthesiology, critical care, and nursing. This results in an environment that has opportunity for both powerful collaboration and powerful conflict. Providers of healthcare should avoid conflict when it has no bearing on outcome, as it is clearly a squandering of individual and collective political capital...
December 2015: Cardiology in the Young
John M Costello, Kathleen Mussatto, Amy Cassedy, Jo Wray, Lynn Mahony, Sarah A Teele, Kate L Brown, Rodney C Franklin, Gil Wernovsky, Bradley S Marino
OBJECTIVE: To determine whether clinicians could reliably predict health-related quality of life (HRQOL) for children with cardiac disease, the level of agreement in predicted HRQOL scores between clinician sub-types, and agreement between clinician-predicted HRQOL scores and patient and parent-proxy reported HRQOL scores. STUDY DESIGN: In this multicenter, cross-sectional study, a random sample of clinical summaries of children with cardiac disease and related patient and parent-proxy reported HRQOL scores were extracted from the Pediatric Cardiac Quality of Life Inventory data registry...
March 2015: Journal of Pediatrics
Richard J Czosek, Amy E Cassedy, Jo Wray, Gil Wernovsky, Jane W Newburger, Kathleen A Mussatto, Lynn Mahony, Ronn E Tanel, Mitchell I Cohen, Rodney C Franklin, Kate L Brown, David Rosenthal, Dennis Drotar, Bradley S Marino
BACKGROUND: Treatment of electrophysiologic (EP) disease in pediatric patients has improved; however, the effects on quality of life (QOL) are unknown. OBJECTIVE: The purpose of this study was to compare QOL within EP disease groups and to other congenital heart diseases, to evaluate the effects of cardiac rhythm devices on QOL, and to identify drivers of QOL in EP disease. METHODS: Cross-sectional study of patient/parent proxy-reported Pediatric Cardiac Quality of Life Inventory scores (Total, Disease Impact, Psychosocial Impact) in subjects aged 8 to 18 years from 11 centers with congenital complete heart block (CCHB), ventricular tachycardia (VT), supraventricular tachycardia (SVT), and long QT syndrome (LQTS)...
May 2015: Heart Rhythm: the Official Journal of the Heart Rhythm Society
Juan Villafañe, M Regina Lantin-Hermoso, Ami B Bhatt, James S Tweddell, Tal Geva, Meena Nathan, Martin J Elliott, Victoria L Vetter, Stephen M Paridon, Lazaros Kochilas, Kathy J Jenkins, Robert H Beekman, Gil Wernovsky, Jeffrey A Towbin
This paper aims to update clinicians on "hot topics" in the management of patients with D-loop transposition of the great arteries (D-TGA) in the current surgical era. The arterial switch operation (ASO) has replaced atrial switch procedures for D-TGA, and 90% of patients now reach adulthood. The Adult Congenital and Pediatric Cardiology Council of the American College of Cardiology assembled a team of experts to summarize current knowledge on genetics, pre-natal diagnosis, surgical timing, balloon atrial septostomy, prostaglandin E1 therapy, intraoperative techniques, imaging, coronary obstruction, arrhythmias, sudden death, neoaortic regurgitation and dilation, neurodevelopmental (ND) issues, and lifelong care of D-TGA patients...
August 5, 2014: Journal of the American College of Cardiology
J William Gaynor, Richard F Ittenbach, Marsha Gerdes, Judy Bernbaum, Robert R Clancy, Donna M McDonald-McGinn, Elaine H Zackai, Gil Wernovsky, Susan C Nicolson, Thomas L Spray
OBJECTIVES: The study objectives were to compare the neurodevelopmental outcomes of preschool survivors of the Fontan procedure with those of children with congenital heart disease undergoing biventricular repair and to investigate predictors of neurodevelopmental outcome for those with single ventricle congenital heart disease, including hypoplastic left heart syndrome. METHODS: Neurodevelopmental outcomes were assessed at 4 years of age, including cognition, visual-motor integration, behavior, social skills, and academic achievement...
April 2014: Journal of Thoracic and Cardiovascular Surgery
Katelyn Mellion, Karen Uzark, Amy Cassedy, Dennis Drotar, Gil Wernovsky, Jane W Newburger, Lynn Mahony, Kathy Mussatto, Mitchell Cohen, Christine Limbers, Bradley S Marino
OBJECTIVES: To compare health-related quality of life (HRQOL) in a group of pediatric patients with congenital heart disease (CHD) and healthy controls and patients with other chronic diseases, and to compare HRQOL among patients with CHD of various severity categories with one another, with controls, and with patients with other chronic diseases. STUDY DESIGN: In this cross-sectional survey, t tests were used to compare patient and proxy-reported Pediatric Quality of Life Inventory 4...
April 2014: Journal of Pediatrics
Amy Cassedy, Dennis Drotar, Richard Ittenbach, Shawna Hottinger, Jo Wray, Gil Wernovsky, Jane W Newburger, Lynn Mahony, Kathleen Mussatto, Mitchell I Cohen, Bradley S Marino
BACKGROUND: Socioeconomic status (SES) is known to influence children's health-related quality of life. Many SES indicators assess distinct dimensions of a family's position rather than measuring the same underlying construct. Many researchers, however, see SES indicators as interchangeable. The primary aim of this study was to determine which measure of SES had the strongest impact on health-related quality of life. METHODS: This is a secondary analysis of the Pediatric Cardiac Quality of Life Inventory Validation Study...
2013: Health and Quality of Life Outcomes
J William Gaynor, Gail P Jarvik, Marsha Gerdes, Daniel S Kim, Ramakrishnan Rajagopalan, Judy Bernbaum, Gil Wernovsky, Susan C Nicolson, Thomas L Spray, Robert R Clancy
OBJECTIVE: The occurrence of an electroencephalographic (EEG) seizure after surgery for complex congenital heart defects has been associated with worse neurodevelopmental (ND) outcomes. We previously identified postoperative seizures documented by 48-hour EEG monitoring in 11% of 178 neonates and infants. Evaluation at 1 year of age did not identify an adverse effect of an EEG seizure on ND outcomes. The current study was undertaken to determine if testing in the preschool period would identify deficits that become apparent as children develop...
July 2013: Journal of Thoracic and Cardiovascular Surgery
Roxanne E Kirsch, Andrew C Glatz, J William Gaynor, Susan C Nicolson, Thomas L Spray, Gil Wernovsky, Geoffrey L Bird
OBJECTIVE: To report practice and outcomes in infants undergoing elective repair of tetralogy of Fallot. METHODS: A review of a retrospective cohort of elective complete repair of infants age 6 months or younger from 1995 to 2009 was performed. Patients were excluded because of previous interventions, hypercyanotic episodes, intensive care admissions, additional major cardiac defects, or if they were not discharged after birth. Length of stay, mortality, and complications were recorded...
February 2014: Journal of Thoracic and Cardiovascular Surgery
Brett R Anderson, Kristen N Stevens, Susan C Nicolson, Stephen B Gruber, Thomas L Spray, Gil Wernovsky, Peter J Gruber
OBJECTIVES: Surgical closure of ventricular septal defects remains the most common pediatric cardiac surgical procedure. No studies, however, have comprehensively analyzed risk factors and drivers of nonmortality outcomes in the current era. The purpose of this study was to assess both baseline characteristics and outcomes of children undergoing surgical repair of ventricular septal defects in a contemporary cohort. METHODS: This retrospective study examined a consecutive series of 369 ventricular septal defect closures at a single institution...
March 2013: Journal of Thoracic and Cardiovascular Surgery
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