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https://www.readbyqxmd.com/read/27645915/a-30-year-experience-with-mixed-type-total-anomalous-pulmonary-venous-connection-a-word-of-caution
#1
Brian Kogon, Jan Fernandez, Subhadra Shashidharan, Kirk Kanter, Bahaaldin Alsoufi
BACKGROUND: Patients with total anomalous pulmonary venous connection can be problematic, particularly those with mixed-type pathology. We aimed to describe a cohort of patients with mixed-type anomalous drainage, highlighting the treatment challenges, and identifying risk factors for poor outcome. METHODS: We reviewed the clinical records of patients who underwent repair for mixed-type total anomalous pulmonary venous connection between 1986 and 2015. RESULTS: A total of 19 patients were identified...
September 20, 2016: Cardiology in the Young
https://www.readbyqxmd.com/read/27597140/-frontiers-in-fontan-failure-a-summary-of-conference-proceedings
#2
Camden Hebson, Wendy Book, Robert W Elder, Ryan Ford, Maan Jokhadar, Kirk Kanter, Brian Kogon, Adrienne H Kovacs, Rebecca D Levit, Michael Lloyd, Kevin Maher, Preeti Reshamwala, Fred Rodriguez, Rene Romero, Thor Tejada, Anne Marie Valente, Gruschen Veldtman, Michael McConnell
"Frontiers in Fontan Failure" was the title of a 2015 conference sponsored by Children's Healthcare of Atlanta and Emory University School of Medicine. In what is hoped to be the first of many such gatherings, speakers and attendees gathered to discuss the problem of long-term clinical deterioration in these patients. Specific focuses included properly defining the problem and then discussing different treatment strategies, both medical and surgical. The health of the liver after Fontan palliation was a particular point of emphasis, as were quality of life and future directions...
September 6, 2016: Congenital Heart Disease
https://www.readbyqxmd.com/read/27592600/palliation-outcomes-of-neonates-born-with-single-ventricle-anomalies-associated-with-aortic-arch-obstruction
#3
Bahaaldin Alsoufi, Courtney McCracken, Subhadra Shashidharan, Brian Kogon, William Border, Kirk Kanter
BACKGROUND: The two most common surgical strategies for the treatment of neonates born with single-ventricle anomalies associated with aortic arch obstruction are the Norwood operation and pulmonary artery banding plus coarctation repair (PAB+COA). We reviewed characteristics and outcomes of neonates who underwent those two surgical strategies at our institution. METHODS: Between 2002 and 2012, 94 neonates with a single ventricle and aortic arch obstruction (excluding hypoplastic left heart syndrome) underwent Norwood (n = 65) or PAB+COA (n = 29)...
September 1, 2016: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/27568146/the-effect-of-noncardiac-and-genetic-abnormalities-on-outcomes-following-neonatal-congenital-heart-surgery
#4
Bahaaldin Alsoufi, Scott Gillespie, William T Mahle, Shriprasad Deshpande, Brian Kogon, Kevin Maher, Kirk Kanter
Significant noncardiac and genetic abnormalities (NC and GA) are common in neonates with congenital heart defects. We sought to examine current-era effect of those abnormalities on early and late outcomes following cardiac surgery. The method from 2002-2012, 1538 neonates underwent repair (n = 860, 56%) or palliation (n = 678, 44%) of congenital heart defects. Regression models examined the effect of NC and GA on operative results, resource utilization, and late outcomes. Neonates with NC and GA (n = 312, 20%) had higher incidence of prematurity (21% vs 13%; P < 0...
2016: Seminars in Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/27530641/extracorporeal-membrane-oxygenator-support-in-infants-with-systemic-pulmonary-shunts
#5
Phil Botha, Shriprasad R Deshpande, Michael Wolf, Micheal Heard, Bahaaldin Alsoufi, Brian Kogon, Kirk Kanter
BACKGROUND: Management of a patent systemic-pulmonary (SP) shunt and the resulting runoff during extracorporeal membrane oxygenation (ECMO) varies among institutions. We have used a strategy of increased flow without surgical reduction of the shunt diameter, and here report our results with this strategy. METHODS: In this database review of 169 successive veno-arterial ECMO runs performed between 2002 and 2013 in infants and neonates, ECMO flow, time to achieve lactate clearance, normal pH, and negative fluid balance were compared in patients with shunts and those without shunts...
September 2016: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/27345094/the-impact-of-dominant-ventricle-morphology-on-palliation-outcomes-of-single-ventricle-anomalies
#6
Bahaaldin Alsoufi, Scott Gillespie, Dennis Kim, Subhadra Shashidharan, Kirk Kanter, Kevin Maher, Brian Kogon
BACKGROUND: Differences in right ventricle and tricuspid valve structure compared with left ventricle and mitral valve structure make them less equipped to support the systemic circulation long term, with subsequent systemic right ventricle failure. We examined the effect of dominant ventricle morphology on single ventricle palliation outcomes. METHODS: We grouped 530 neonates who underwent first-stage palliation into two groups based on dominant ventricle morphology: right dominant ventricle (RV group; n = 302, 57%) and left dominant ventricle or functional single ventricle with two well-formed ventricles (LV group; n = 228, 43%)...
August 2016: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/27207392/current-outcomes-of-surgical-management-of%C3%A2-aortopulmonary-window-and-associated-cardiac-lesions
#7
Bahaaldin Alsoufi, Brian Schlosser, Courtney McCracken, Brian Kogon, Kirk Kanter, William Border, Ritu Sachdeva
BACKGROUND: Aortopulmonary window (APW) is a rare congenital defect that is often associated with other cardiac lesions. We analyzed our operative strategy to determine whether this had any relationship with outcomes. METHODS: Early and late outcomes of 40 children who underwent APW repair at our institution during a 20-year period (1994 to 2013) were analyzed. RESULTS: Median age at time of the operation was 22 days (interquartile range, 9 to 63 days), and median weight was 3...
August 2016: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/27174514/outcomes-of-neonates-requiring-prolonged-stay-in-the-intensive-care-unit-after-surgical-repair-of-congenital-heart-disease
#8
Makoto Mori, Courtney McCracken, Kevin Maher, Brian Kogon, William Mahle, Kirk Kanter, Bahaaldin Alsoufi
OBJECTIVE: After neonatal cardiac surgery, a number of patients need a prolonged stay in the intensive care unit (ICU). Those patients require tremendous resources and strain the capacity of cardiac units. To date, little knowledge of early and late survival for this challenging population exists. METHODS: From 2002 to 2012, 108 neonates required a postoperative ICU stay >30 days. Multivariable regression analyses examined factors associated with hospital death and late survival...
September 2016: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/27151913/age-dependent-effect-of-pediatric-cardiac-progenitor-cells-after-juvenile-heart-failure
#9
Udit Agarwal, Amanda W Smith, Kristin M French, Archana V Boopathy, Alex George, David Trac, Milton E Brown, Ming Shen, Rong Jiang, Janet D Fernandez, Brian E Kogon, Kirk R Kanter, Baahaldin Alsoufi, Mary B Wagner, Manu O Platt, Michael E Davis
UNLABELLED: Children with congenital heart diseases have increased morbidity and mortality, despite various surgical treatments, therefore warranting better treatment strategies. Here we investigate the role of age of human pediatric cardiac progenitor cells (hCPCs) on ventricular remodeling in a model of juvenile heart failure. hCPCs isolated from children undergoing reconstructive surgeries were divided into 3 groups based on age: neonate (1 day to 1 month), infant (1 month to 1 year), and child (1 to 5 years)...
July 2016: Stem Cells Translational Medicine
https://www.readbyqxmd.com/read/27085618/outcomes-of-multistage-palliation-of-infants-with-functional-single-ventricle-and-heterotaxy-syndrome
#10
COMPARATIVE STUDY
Bahaaldin Alsoufi, Courtney McCracken, Brian Schlosser, Ritu Sachdeva, Andrew Well, Brian Kogon, William Border, Kirk Kanter
BACKGROUND: Management of infants with heterotaxy syndrome and functional single ventricle is complicated due to associated cardiac and extracardiac anomalies. We report current era palliation results. METHODS: Between 2002 and 2012, 67 infants with heterotaxy syndrome underwent multistage palliation. Competing risks analyses modeled events after surgery (death vs Glenn procedure) and examined factors associated with survival. In addition, early and late outcomes following first-stage palliation surgery were compared with a matched contemporaneous control group of patients with nonheterotaxy single ventricle anomalies...
May 2016: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/27033246/superior-mesenteric-arterial-flow-pattern-is-associated-with-major-adverse-events-in-adults-with-fontan-circulation
#11
Makoto Mori, Kayoko Shioda, Robert W Elder, Maria A Pernetz, Fred H Rodriguez, Alicia Rangosch, Brian E Kogon, Wendy M Book
Factors contributing to the failure of Fontan circulation in adults are poorly understood. Reduced superior mesenteric arterial (SMA) flow has been identified in pediatric Fontan patients with protein-losing enteropathy. SMA flow has not been profiled in an adult Fontan population and its association with adverse events is unknown. We aimed to examine associations between SMA flow patterns and adverse events in adult Fontan patients. We performed a retrospective review of adult Fontan patients who underwent echocardiograms between 2008 and 2014...
August 2016: Pediatric Cardiology
https://www.readbyqxmd.com/read/27032470/era-effect-on-survival-following-paediatric-heart-transplantation
#12
Bahaaldin Alsoufi, Shriprasad Deshpande, Courtney McCracken, Brian Kogon, Robert Vincent, William T Mahle, Kirk Kanter
OBJECTIVES: Paediatric heart transplantation is performed to salvage children with end-stage heart failure from various underlying pathologies. Despite several developments in all management aspects of transplantation candidates, the effect of those advances on outcomes has not been consistent. We report our institutional experience with focus on examining era effect on early and late survival. METHODS: Between 1988 and 2015, 320 children underwent heart transplantation...
October 2016: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/27002229/congenital-cardiac-surgery-fellowship-training-a-status-update
#13
Brian Kogon, Tara Karamlou, William Baumgartner, Walter Merrill, Carl Backer
BACKGROUND: In 2007, congenital cardiac surgery became a recognized fellowship by the Accreditation Council of Graduate Medical Education (ACGME) and leads to board certification through the American Board of Thoracic Surgery (ABTS). We highlight the strengths and weaknesses in the current system of accredited training. METHODS: Data were collected from program directors, the ACGME, and the ABTS. In addition, surveys were sent to training program graduates. Topics included program accreditation status, number of fellows trained per year and per program, match results, fellow operative experience, fellow satisfaction, and post-fellowship employment status...
June 2016: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/26932151/catheter-measured-hemodynamics-of-adult-fontan-circulation-associations-with-adverse-event-and-end-organ-dysfunctions
#14
Makoto Mori, Camden Hebson, Kayoko Shioda, Robert W Elder, Brian E Kogon, Fred H Rodriguez, Maan Jokhadar, Wendy M Book
BACKGROUND: In heart failure, a high systemic vascular resistance index (SVRI), high central venous pressure (CVP), and low cardiac index (CI) predict poor outcomes. Conversely, late hemodynamic manifestations of failing Fontan circulation and associations with end-organ dysfunction are not well understood. METHODS: A retrospective review of right-heart catheterization data of adult Fontan patients between 2002 and 2014 was conducted. Relationships between hemodynamic variables and serious adverse events (death or heart transplant) were examined using the Cox proportional hazard analysis...
December 2016: Congenital Heart Disease
https://www.readbyqxmd.com/read/26912652/factors-affecting-death-and-progression-towards-next-stage-following-modified-blalock-taussig-shunt-in-neonates
#15
Bahaaldin Alsoufi, Scott Gillespie, Makoto Mori, Martha Clabby, Kirk Kanter, Brian Kogon
OBJECTIVES: The modified Blalock-Taussig shunt (BTS) is utilized to palliate neonates born with restrictive pulmonary blood flow including those with single ventricle (SV) or biventricular (BV) cardiac anomalies. We aim in the current study to report palliation outcomes of neonates with BTS and to examine factors affecting death and progression to the subsequent stage of palliation or repair. METHODS: Between 2002 and 2012, 341 patients underwent BTS including 175 with SV and 166 with BV anomalies...
July 2016: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/26803993/late-primary-transplantation-in-complex-congenital-heart-disease
#16
Shriprasad R Deshpande, Bahig M Shehata, Denver Sallee, Brian E Kogon, William T Mahle
Heart transplantation is a viable option for patients with congenital heart disease who manifest heart failure, predominantly post-surgical palliation. We present a case of complex congenital heart disease, where surgical palliation was considered not to be an option. Considerate medical management with close follow-up allowed for a nine-yr delay until eventual successful heart transplantation. The case also highlights the natural history over nine yr of the effects of right ventricular outflow tract obstruction on the left ventricular remodeling which is analogous to a PA banding in patients with dilated cardiomyopathy...
March 2016: Pediatric Transplantation
https://www.readbyqxmd.com/read/26762915/transcatheter-versus-surgical-closure-of-atrial-septal-defects-in-children-a-value-comparison
#17
Yinn Khurn Ooi, Michael Kelleman, Alexandra Ehrlich, Michelle Glanville, Arlene Porter, Dennis Kim, Brian Kogon, Matthew E Oster
OBJECTIVES: The purpose of this study was to determine whether a transcatheter procedure or surgical closure offers a better value proposition for atrial septal defect (ASD) closure. BACKGROUND: Secundum ASDs are common congenital heart defects with both transcatheter and surgical treatment options. Although both options have been shown to have excellent results in children, the relative value of the 2 procedures is unclear. METHODS: Using data from the Pediatric Hospital Information System for 2004 to 2012, we compared the value of transcatheter versus surgical ASD closure for children ages 1 to 17 years, with value being defined as outcomes relative to costs...
January 11, 2016: JACC. Cardiovascular Interventions
https://www.readbyqxmd.com/read/26520008/outcomes-of-heart-transplantation-in-children-with-hypoplastic-left-heart-syndrome-previously-palliated-with-the-norwood-procedure
#18
Bahaaldin Alsoufi, William T Mahle, Cedric Manlhiot, Shriprasad Deshpande, Brian Kogon, Brian W McCrindle, Kirk Kanter
BACKGROUND: Following the Norwood operation, unfavorable hemodynamic or anatomic factors might disqualify children from progressing through subsequent palliative surgeries necessitating listing for heart transplantation. Those patients often have immune, clinical, or anatomic risk factors that could preclude donor allocation, increase operative risk, and diminish late survival. We studied transplantation outcomes in those patients using the Pediatric Heart Transplant Study database. METHODS: A total of 253 children who had prior Norwood were listed for transplantation (1993-2012)...
January 2016: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/26520007/selective-management-strategy-of-interrupted-aortic-arch-mitigates-left-ventricular-outflow-tract-obstruction-risk
#19
Bahaaldin Alsoufi, Brian Schlosser, Courtney McCracken, Ritu Sachdeva, Brian Kogon, William Border, William T Mahle, Kirk Kanter
BACKGROUND: Left ventricular outflow tract obstruction (LVOTO) is an important problem after interrupted aortic arch (IAA) repair, especially when early reoperation is required during infancy. Several anatomic factors have been identified that increase LVOTO risk; surgical strategies such as concomitant resection of the conal septum or left ventricular outflow tract (LVOT) bypass (single-stage Yasui operation, or staged Norwood procedure, followed by the Rastelli procedure) have been proposed for such patients...
February 2016: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/26505290/depressive-symptoms-in-children-with-chronic-kidney-disease
#20
Amy J Kogon, Matthew B Matheson, Joseph T Flynn, Arlene C Gerson, Bradley A Warady, Susan L Furth, Stephen R Hooper
OBJECTIVE: To assess depression in children with chronic kidney disease and to determine associations with patient characteristics, intellectual and educational levels, and health-related quality of life (HRQoL). STUDY DESIGN: Subjects aged 6-17 years from the Chronic Kidney Disease in Children cohort study completed the Children's Depression Inventory (CDI), Wechsler Abbreviated Scales of Intelligence, Wechsler Individual Achievement Test-II-Abbreviated, and the Pediatric Inventory of Quality of Life Core Scales 4...
January 2016: Journal of Pediatrics
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