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Children's Mercy Kansas City

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Articles 1 - 30 of 244

Full-Text Articles in Cardiology

How To Assess Ventricular Function By Fetal Echocardiography: Expert Guidance From The Fetal Heart Society., S R Patel, Nitin Madan, L Eckersley, Z Roytman, M Perez, L Gindes, L K Hornberger Jul 2026

How To Assess Ventricular Function By Fetal Echocardiography: Expert Guidance From The Fetal Heart Society., S R Patel, Nitin Madan, L Eckersley, Z Roytman, M Perez, L Gindes, L K Hornberger

Manuscripts, Articles, Book Chapters and Other Papers

No abstract provided.


A 2-Year Randomized Trial Of Pitavastatin Calcium Vs Placebo To Treat Combined Dyslipidemia In Adolescents With Overweight And Obesity., Brian W. Mccrindle, Silva Arslanian, Michele Mietus-Snyder, Elaine M. Urbina, Adam L. Ware, Jessica E. Teng, Felicia Trachtenberg, Mark W. Russell, Amy S. Shah, Mark Cartoski, Julie A. Brothers, Christine San Giovanni, Justin P. Zachariah, Sandra Pena, William T. Mahle, Amy L. Peterson, Sheela N. Magge, Geetha Raghuveer, Binu Sharma, Valiantsina Kazlova, Craig A. Sponseller, D'Andrea Freemon, Mario Stylianou, Sarah D. De Ferranti, Pediatric Heart Network Investigators Jun 2026

A 2-Year Randomized Trial Of Pitavastatin Calcium Vs Placebo To Treat Combined Dyslipidemia In Adolescents With Overweight And Obesity., Brian W. Mccrindle, Silva Arslanian, Michele Mietus-Snyder, Elaine M. Urbina, Adam L. Ware, Jessica E. Teng, Felicia Trachtenberg, Mark W. Russell, Amy S. Shah, Mark Cartoski, Julie A. Brothers, Christine San Giovanni, Justin P. Zachariah, Sandra Pena, William T. Mahle, Amy L. Peterson, Sheela N. Magge, Geetha Raghuveer, Binu Sharma, Valiantsina Kazlova, Craig A. Sponseller, D'Andrea Freemon, Mario Stylianou, Sarah D. De Ferranti, Pediatric Heart Network Investigators

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Combined dyslipidemia of overweight/obesity (CDO) is prevalent in youth and is associated with an increased risk of early cardiovascular disease.

OBJECTIVE: We sought to determine the impact and safety of statin therapy for CDO in adolescents.

METHODS: A double-blind, randomized trial was performed across 18 North American sites. Participants aged 10 to 19 years with body mass index ≥85th percentile and CDO defined as non-high-density lipoprotein cholesterol (non-HDL-C) ≥120 mg/dL (3.10 mmol/L) and either low HDL-C or high triglyceride:HDL-C ratio were randomized centrally to receive either pitavastatin calcium 4 mg/d or placebo for 2 years. The primary outcome was …


Practice Patterns In Pediatric Cardiothoracic Presurgical Conferences: A Multicenter Survey Study., Britney Reed, Poonam Puranik, Andrew Rodenbarger, Mira Trivedi, Dilachew A. Adebo, Gary Beasley, Louis Bezold, M Jay Campbell, Michael R. Carr, Joshua Daily, Mark Debrunner, Erica Del Grippo, Carlen G. Fifer, Timothy M. Hoffman, Susan R. Hupp, Joshua D. Kurtz, Gira Morchi, Carl Owada, Renuka Peterson, Michael D. Puchalski, Ryan Romans, Arwa Saidi, Rajesh U. Shenoy, Anoop K. Singh, Robert D. Tunks, Thomas M. Yohannan, Carolyn M. Wilhelm, Jyoti K. Patel Jun 2026

Practice Patterns In Pediatric Cardiothoracic Presurgical Conferences: A Multicenter Survey Study., Britney Reed, Poonam Puranik, Andrew Rodenbarger, Mira Trivedi, Dilachew A. Adebo, Gary Beasley, Louis Bezold, M Jay Campbell, Michael R. Carr, Joshua Daily, Mark Debrunner, Erica Del Grippo, Carlen G. Fifer, Timothy M. Hoffman, Susan R. Hupp, Joshua D. Kurtz, Gira Morchi, Carl Owada, Renuka Peterson, Michael D. Puchalski, Ryan Romans, Arwa Saidi, Rajesh U. Shenoy, Anoop K. Singh, Robert D. Tunks, Thomas M. Yohannan, Carolyn M. Wilhelm, Jyoti K. Patel

Manuscripts, Articles, Book Chapters and Other Papers

Pediatric cardiothoracic surgeries are high-stakes, complex procedures, typically undergoing prior review at multidisciplinary conferences. This study evaluates practice patterns of conferences throughout the United States (US). Surveys were distributed to fellowship program directors or division directors in 124 US pediatric cardiology centers seeking information on conference logistics, fellow roles, quality improvement (QI), and satisfaction. All 47 responding centers (response rate 38%) conduct presurgical conferences, mostly on a weekly basis (92%) lasting 60-120 min (79%). The conferences are solely virtual (19%) or hybrid (81%). High-volume centers (> 300 surgical cases/year) are more likely to hold multiple conferences (13/20 vs 7/27, p <  0.01) and less likely to designate a moderator (11/20 vs 22/26, p = 0.027). Categorical pediatric cardiology fellows at 33 centers present clinical data (97%), echocardiograms (85%), catheterizations (82%), and cross-sectional imaging (39%), typically beginning in their first year. Most centers report that minor (98%) or major changes (51%) are made to patient management at least "sometimes." Responders rate conferences as very important (median 10/10 on a 10-point Likert scale, IQR 9-10), but satisfaction is more modest (median 7/10, IQR 7-9). Only 17% of centers have a formal QI process. Comments from 42 centers reveal positive themes of collaboration (68%) but also concerns about lengthy (30%) or inefficient (36%) discussion. Conclusions: This survey highlights common practices for pediatric cardiothoracic presurgical conferences. Conferences are collaborative and seen as highly impactful. However, satisfaction varies, and QI efforts are infrequent. These findings highlight opportunities for process improvement and standardization.


Morphological Assessment Of Stage Hh38 Of The Japanese Quail (Coturnix Japonica) Heart By Micro-Sonogram, Jaden Roe, Ashlyn Benavides, Michael B. Filla, Douglas C. Bittel, Whitney Shae, Geetha Haligheri, James E. O'Brien, Nataliya Kibiryeva May 2026

Morphological Assessment Of Stage Hh38 Of The Japanese Quail (Coturnix Japonica) Heart By Micro-Sonogram, Jaden Roe, Ashlyn Benavides, Michael B. Filla, Douglas C. Bittel, Whitney Shae, Geetha Haligheri, James E. O'Brien, Nataliya Kibiryeva

Manuscripts, Articles, Book Chapters and Other Papers

A challenge of studying mammalian cardiac embryogenesis is the limited ability to perform experimental manipulations in animal models. The avian embryo is widely accepted as a model for mammalian heart developmental studies. In this study, we establish the methodology and protocols for studying the Japanese quail (Coturnix japonica) heart at embryonic day 10 (HH38) using the FUJIFILM VisualSonics Vevo 3100 ultrasound system equipped with a MX550D small animal cardiology transducer. These protocols were designed to measure right ventricular wall thickness, pulmonary artery diameter, and the outflow velocities of the right ventricular outflow tract (RVOT) and the pulmonary artery …


Protocol For Non-Invasive Assessment Of Skeletal Muscle Structure And Function In Adolescents With Single Ventricle Heart Disease: A Cross-Sectional, Case-Control Study., Tyler A. Fick, Trent J. Herda, Emily Cramer, David A. White, Daniel Forsha Apr 2026

Protocol For Non-Invasive Assessment Of Skeletal Muscle Structure And Function In Adolescents With Single Ventricle Heart Disease: A Cross-Sectional, Case-Control Study., Tyler A. Fick, Trent J. Herda, Emily Cramer, David A. White, Daniel Forsha

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Congenital heart disease (CHD) is the most common congenital malformation, and the most severe type, single ventricle (SV) heart disease, requires the Fontan surgical palliation. Fontan palliation minimizes hypoxemia and volume overload by separating systemic and pulmonary circulations, resulting in the absence of a sub-pulmonary pump. Skeletal muscle health may have a greater relative contribution to overall functional capacity of those with SV physiology, via the skeletal muscle (SkM) pump mechanism and oxidative capacity. The purpose of this study is to comprehensively evaluate multiple domains of SkM in this population compared to those with normal cardiac anatomy.

METHODS: Forty …


Cardiac Medication Use In Action For Duchenne Muscular Dystrophy Cardiomyopathy., Carol A. Wittlieb-Weber, Brian Birnbaum, Chesney D. Castleberry, Tyler W. Cunningham, Paul Esteso, Katheryn E. Gambetta, Emily A. Hayes, Daphne T. Hsu, Beth D. Kaufman, Benjamin Kroslowitz, Ashwin K. Lal, Angela Lorts, Hugo Martinez, Deepa Mokshagundam, Deipanjan Nandi, John J. Parent, Frank Raucci, Nelia Soares, Jonathan H. Soslow, Renata Shih, Svetlana Shugh, Chet R. Villa, Sarah J. Wilkens, Bethany L. Wisotzkey, Jennifer Conway Apr 2026

Cardiac Medication Use In Action For Duchenne Muscular Dystrophy Cardiomyopathy., Carol A. Wittlieb-Weber, Brian Birnbaum, Chesney D. Castleberry, Tyler W. Cunningham, Paul Esteso, Katheryn E. Gambetta, Emily A. Hayes, Daphne T. Hsu, Beth D. Kaufman, Benjamin Kroslowitz, Ashwin K. Lal, Angela Lorts, Hugo Martinez, Deepa Mokshagundam, Deipanjan Nandi, John J. Parent, Frank Raucci, Nelia Soares, Jonathan H. Soslow, Renata Shih, Svetlana Shugh, Chet R. Villa, Sarah J. Wilkens, Bethany L. Wisotzkey, Jennifer Conway

Manuscripts, Articles, Book Chapters and Other Papers

This study seeks to understand cardiac medication use in a large cohort of males with Duchenne Muscular Dystrophy (DMD) followed prospectively with focus on current practices and adherence to consensus directed medical therapy (CDMT). DMD patients have been enrolled in the Advanced Cardiac Therapies Improving Outcomes Network (ACTION) Dystrophinopathy Registry since 2021. Cardiac medication use was analyzed at enrollment and most recent follow-up. CDMT was defined as concurrent use of angiotensin-converting-enzyme inhibitor (ACEi)/angiotensin II receptor blocker (ARB) /angiotensin receptor-neprilysin inhibitor (ARNI) plus beta-blocker (BB) plus mineralocorticoid receptor antagonist (MRA). Two hundred sixty-five males with DMD (median age 17.5 (IQR 14.5-21.5) …


Hyperacute Effects Of Non-Code Dose Bolus Epinephrine In Paediatric Cardiac Intensive Care Patients: Insights From High-Fidelity Physiologic Data., Wesam Sourour, Michael Evans, Kevin Le, Saul Flores, Juan S. Farias, Rohit Seth Loomba Mar 2026

Hyperacute Effects Of Non-Code Dose Bolus Epinephrine In Paediatric Cardiac Intensive Care Patients: Insights From High-Fidelity Physiologic Data., Wesam Sourour, Michael Evans, Kevin Le, Saul Flores, Juan S. Farias, Rohit Seth Loomba

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Non-code dose boluses of epinephrine are utilised in critically ill paediatric patients during periods of hemodynamic deterioration, often with the hopes of preventing a cardiac arrest. Data regarding the physiologic effects of these administrations are limited. The primary aim of this study was to use high-fidelity physiologic data to characterise the effects of intravenous non-code dose bolus epinephrine.

METHODS: Paediatric patients in the cardiac ICU who received non-code dose bolus epinephrine were identified. Those who received fluid boluses or chest compressions within 2 minutes of bolus epinephrine were excluded. Autoregressive integrated moving average analyses with exogenous variables were conducted …


Quantitative Echocardiography Markers To Denote Severity Of Tricuspid Regurgitation Compared To Cardiac Mri In Single Right Ventricle Variants, Tony Zunica, Daniel Forsha, Janelle Noel-Macdonnell, Anmol Goyal Feb 2026

Quantitative Echocardiography Markers To Denote Severity Of Tricuspid Regurgitation Compared To Cardiac Mri In Single Right Ventricle Variants, Tony Zunica, Daniel Forsha, Janelle Noel-Macdonnell, Anmol Goyal

Posters

Background: Single right ventricle and Hypoplastic Left Heart Syndrome (HLHS) are congenital heart diseases that require multiple palliative surgeries to create a single right ventricular system. 25-30% of these patients develop significant tricuspid regurgitation (TR), which is associated with worse outcomes among all stages of palliation. Echocardiography remains the cornerstone imaging modality for serial evaluation in these patients. However, estimating tricuspid regurgitation is highly subjective in the pediatric population, necessitating need for reliable and reproducible quantitative echocardiography markers. Currently, cardiac magnetic resonance (CMR) is considered the gold standard for quantification of tricuspid regurgitation with good accuracy and reproducibility. Objectives/Goal: Our …


Diagnostic Challenges Of Anomalous Left Coronary Artery From The Right Pulmonary Artery., Lavina Desai, Sanket Shah, Edo Bedzra, Christopher Mathis Feb 2026

Diagnostic Challenges Of Anomalous Left Coronary Artery From The Right Pulmonary Artery., Lavina Desai, Sanket Shah, Edo Bedzra, Christopher Mathis

Manuscripts, Articles, Book Chapters and Other Papers

• ALCAPA is a rare congenital heart defect seen in about one in 300,000 live births. • ALCARPA is an even rarer subset of ALCAPA with the LCA originating from the RPA. • Few cases report ALCARPA associated with coarctation of the aorta. • Coarctation and VSD may cause PH with antegrade LCA flow. • These defects are hard to diagnose and require coronary imaging before surgery.


Left And Right Ventricular Strain Mechanics In Fetal Tachyarrhythmia., Rukmini Komarlu, Janelle R. Noel-Macdonnell Phd, Neha Chellu, Geetha Haligheri Dec 2025

Left And Right Ventricular Strain Mechanics In Fetal Tachyarrhythmia., Rukmini Komarlu, Janelle R. Noel-Macdonnell Phd, Neha Chellu, Geetha Haligheri

Manuscripts, Articles, Book Chapters and Other Papers

Introduction: Assessment of myocardial function in fetuses with supraventricular tachyarrhythmia is challenging. Speckle-tracking echocardiography (STE) is a newer sensitive method to assess ventricular systolic function. We sought to assess left (LV) and right (RV) ventricular myocardial strain mechanics in fetuses with tachyarrhythmia and hypothesized that strain mechanics are impaired in this patient population even after conversion to sinus rhythm.

METHODS: This was a single-center retrospective review. LV and RV strain parameters were assessed using STE in tachyarrhythmia and after conversion to sinus rhythm and, compared to gestational age (GA), matched control fetuses in sinus rhythm.

RESULTS: Eighteen fetuses with tachyarrhythmia …


Perioperative Nutrition In Pediatric Patients With Congenital Heart Disease And Heart Failure., Jaime Silva-Gburek, Kelsey May, Bailey Walvoord, Catalina Lozano, Jorge A. Coss-Bu Nov 2025

Perioperative Nutrition In Pediatric Patients With Congenital Heart Disease And Heart Failure., Jaime Silva-Gburek, Kelsey May, Bailey Walvoord, Catalina Lozano, Jorge A. Coss-Bu

Manuscripts, Articles, Book Chapters and Other Papers

Optimal nutritional therapy is important for infants and children with perioperative congenital heart disease and heart failure. Medical providers face physiological and metabolic challenges when administering enteral and parenteral nutrition to these patients. Complications related to enteral nutrition can increase morbidity and mortality, although outcomes are improved in those cases in which caloric and nutrient administration can be achieved. Consensus regarding feeding strategies and timing of nutritive care is lacking. This narrative review aims to summarize, analyze and discuss the most recent literature on nutritional therapy in perioperative congenital heart disease, heart failure and pediatric patients requiring mechanical circulatory support …


Short- And Mid-Term Outcomes Of The Extravascular Implantable Cardioverter-Defibrillator In Pediatric And Adolescent Patients: A Single-Center Experience., Nikola Dragisic, Emily R. Backes, Lindsey Malloy-Walton, Edo Bedzra, William Gibson, Philip M. Chang Nov 2025

Short- And Mid-Term Outcomes Of The Extravascular Implantable Cardioverter-Defibrillator In Pediatric And Adolescent Patients: A Single-Center Experience., Nikola Dragisic, Emily R. Backes, Lindsey Malloy-Walton, Edo Bedzra, William Gibson, Philip M. Chang

Manuscripts, Articles, Book Chapters and Other Papers

Experience with the Aurora extravascular (EV) implantable cardioverter-defibrillator (ICD) (Medtronic, Minneapolis, MN, USA) remains limited among pediatric centers in contrast to its broader implantation in adults. We evaluated our single-center experience with the EV ICD as part of our standard ICD options for treatment of malignant ventricular arrhythmias in pediatric subjects. Following approval for EV ICD implantation at our center, five consecutive adolescent patients underwent implantation over a 7-month period. Patient characteristics, qualification for EV ICD placement, nuances in EV ICD implantation in each patient, and post-implant follow-up were reviewed. A total of six out of eight consecutive ICD candidates …


Use And Outcomes Of The Medical Hybrid Procedure For Stage 1 Palliation In Infants With Hypoplastic Left Heart Syndrome And Variants., Daniel N. Beauchamp, Christopher J. Statile, Huaiyu Zang, David A. Parra, Justin Godown, Natalie Jayaram, Matthew L. Moehlmann, Garick D. Hill Nov 2025

Use And Outcomes Of The Medical Hybrid Procedure For Stage 1 Palliation In Infants With Hypoplastic Left Heart Syndrome And Variants., Daniel N. Beauchamp, Christopher J. Statile, Huaiyu Zang, David A. Parra, Justin Godown, Natalie Jayaram, Matthew L. Moehlmann, Garick D. Hill

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Staged palliation of hypoplastic left heart syndrome and variants begins with the Norwood or hybrid procedure. Hybrid palliation is used in a minority of cases and often reserved for high-risk patients. Stented hybrid (SH) comprises bilateral pulmonary artery bands and ductal stenting, and medical hybrid (MH) comprises bilateral pulmonary artery bands and prostaglandins. MH use and outcomes have not been well described. We sought to compare MH, SH, and surgical stage 1 (SS1) using a national database.

METHODS: Patients from the National Pediatric Cardiology Quality Improvement Collaborative database born between 2016 and 2021 were categorized by initial intervention: MH, …


Zero Fluoroscopy Ablation Of Arrhythmias In Patients With Congenital Heart Disease, Shailendra Upadhyay, Jenna Schermerhorn, Whitney Fairchild, Jamie Bopp, Irfan Warsy Nov 2025

Zero Fluoroscopy Ablation Of Arrhythmias In Patients With Congenital Heart Disease, Shailendra Upadhyay, Jenna Schermerhorn, Whitney Fairchild, Jamie Bopp, Irfan Warsy

Manuscripts, Articles, Book Chapters and Other Papers

Aims

Radiation-free catheter ablation is feasible with modern electroanatomic mapping systems. We aimed to evaluate the feasibility, safety, and outcomes of non-fluoroscopic ablation (NFA) in patients with congenital heart disease (CHD).

Methods

We retrospectively reviewed CHD patients who underwent NFA between November 2016 and January 2025. All procedures were performed using the CARTO 3D electroanatomic mapping system. Atrial, ventricular, and aortic geometries were reconstructed as needed. Catheter navigation and sheath placement were guided without fluoroscopy; intracardiac echocardiography was used selectively.

Results

Forty-two patients (23 females) with CHD underwent NFA. The median age was 14 years (range 4–56), and median weight …


Intravenous Sotalol In Pediatric Cardiac Intensive Care Patients Compared To Other Antiarrhythmics: Insights From A Multicenter Database, Rohit S. Loomba, Emily R. Backes, Juan Farias Torres, Alejandro Borquez, Lindsey Malloy-Walton, Saul Flores Nov 2025

Intravenous Sotalol In Pediatric Cardiac Intensive Care Patients Compared To Other Antiarrhythmics: Insights From A Multicenter Database, Rohit S. Loomba, Emily R. Backes, Juan Farias Torres, Alejandro Borquez, Lindsey Malloy-Walton, Saul Flores

Posters

Background: Sotalol is an antiarrhythmic for which an intravenous (IV) form became available in 2015. Studies suggest it is safe and effective for treating pediatric arrhythmias, with fewer effects on physiologic parameters and cardiac output than amiodarone, and it does not appear to be associated with its long-term risks. However, data in pediatric patients remains limited. This study aimed to characterize the use of intravenous sotalol in pediatric intensive care admissions and compare its use and outcomes with intravenous amiodarone and procainamide. Methods: Data were drawn from the Pediatric Health Information System across six hospitals with the highest IV sotalol …


Fontan Circulation And Transplantation: Considerations For The Complex Candidate., Drishti Tolani, Sharon Chen, Edo Bedzra, Kurt R. Schumacher, Shahnawaz Amdani Nov 2025

Fontan Circulation And Transplantation: Considerations For The Complex Candidate., Drishti Tolani, Sharon Chen, Edo Bedzra, Kurt R. Schumacher, Shahnawaz Amdani

Manuscripts, Articles, Book Chapters and Other Papers

The Fontan operation has transformed survival for patients with single ventricle congenital heart disease, but the long-term durability of this physiology remains limited. Fontan circulatory failure (FCF) is a progressive, heterogeneous condition associated with multiorgan dysfunction, lymphatic failure, and elevated post-transplant risk when not recognized early. With increasing survival into adulthood, heart transplantation has emerged as the definitive "fourth stage" of palliation. However, referrals for advanced therapies are often delayed, with many patients presenting in advanced stages of decline. This review synthesizes current evidence on the pathophysiology of Fontan failure, outlines consensus indications for transplant evaluation, and highlights pre-transplant strategies …


Fetal Congenital Complete Heart Block: 2 Success Stories Through Multidisciplinary Care., Mohamed Aashiq Abdul Ghayum, Jenna Schermerhorn, Hayley S. Hancock, Lindsey Malloy-Walton, Steven Olsen, Laura Vricella, Kelsey Brattrud, Melanie Kathol, Rita France, Maria Kiaffas Sep 2025

Fetal Congenital Complete Heart Block: 2 Success Stories Through Multidisciplinary Care., Mohamed Aashiq Abdul Ghayum, Jenna Schermerhorn, Hayley S. Hancock, Lindsey Malloy-Walton, Steven Olsen, Laura Vricella, Kelsey Brattrud, Melanie Kathol, Rita France, Maria Kiaffas

Manuscripts, Articles, Book Chapters and Other Papers

Congenital complete heart block (CCHB) is a rare condition associated with significant fetal and neonatal mortality. It may present as an isolated conduction abnormality in association with autoimmune disease, or in conjunction with congenital heart defects including heterotaxy-polysplenia syndrome. Early prenatal diagnosis and coordinated perinatal care are critical for improving outcomes. We present 2 cases of prenatally diagnosed CCHB. The first occurred in a fetus with heterotaxy-polysplenia syndrome, and the second in the setting of maternal anti-SSA antibody exposure. Both fetuses developed progressive bradycardia, with heart rates declining to < 50 beats/min, necessitating close fetal surveillance and individualized perinatal management. Preterm delivery and prompt placement of temporary pacing wires allowed initial stabilization before eventual definitive management, resulting in survival. The successful outcomes in these neonates highlight the critical role of multidisciplinary prenatal care, timely delivery planning, and early postnatal intervention in improving survival in fetuses diagnosed with CCHB.


Semilunar Valve Replacement With A Telescoping Arterial Trunk Valve, Edo Bedzra, Herra Javed, James E. O'Brien, Taufiek Konrad Rajab Sep 2025

Semilunar Valve Replacement With A Telescoping Arterial Trunk Valve, Edo Bedzra, Herra Javed, James E. O'Brien, Taufiek Konrad Rajab

Manuscripts, Articles, Book Chapters and Other Papers

A bicuspid aortic valve, from autologous tissue, with growth potential can be constructed using the simple, and reproducible telescoping arterial trunk technique.


Association Between Chd And Cerebral Abscess: Insights From A National Database., Keats Ewing, Rohit Seath Loomba, Saul Flores, Enrique G. Villarreal, Juan S. Farias, Matthew Taylor Coghill Sep 2025

Association Between Chd And Cerebral Abscess: Insights From A National Database., Keats Ewing, Rohit Seath Loomba, Saul Flores, Enrique G. Villarreal, Juan S. Farias, Matthew Taylor Coghill

Manuscripts, Articles, Book Chapters and Other Papers

CHD has been historically associated with the development of cerebral abscess. This retrospective cohort study examines the association of CHD with the occurrence of cerebral abscess in an inpatient paediatric population. We analysed data from the Pediatric Health Information System database, an administrative database that captures data from participating children's hospitals in the United States. We included all patients admitted to participating paediatric ICUs between 2016 and 2021 for a total of 426,029 admissions. Including all admissions, 1,387 (0.3%) patients experienced a cerebral abscess, and of 80,927 (19%) patients with CHD, only 88 (0.1%) experienced a cerebral abscess (odds ratio …


Revisiting The Effect Of Udenafil On Exercise Performance In Patients With Fontan Circulation: Results Of A Post Hoc Analysis Of The Fuel Trial., David J. Goldberg, Victor Zak, Chenwei Hu, Adam M. Lubert, Rahul H. Rathod, Daniel J. Penny, Robert Whitehill, Kurt R. Schumacher, Salil Ginde, Michelle Ploutz, Gi Beom Kim, Todd T. Nowlen, Michael V. Dimaria, Benjamin P. Frischhertz, Jonathan B. Wagner, Kimberly E. Mchugh, Brian W. Mccrindle, Mark J. Cartoski, Jon Detterich, Anji T. Yetman, Anitha S. John, Marc E. Richmond, Matthew Files, R Mark Payne, Andrew S. Mackie, Christopher K. Davis, William B. Orr, Ruchira Garg, Kevin D. Hill, Jennifer S. Yauck, Michael G. Mcbride, James Yeager, Gail D. Pearson, Stephen M. Paridon, Bryan H. Goldstein Aug 2025

Revisiting The Effect Of Udenafil On Exercise Performance In Patients With Fontan Circulation: Results Of A Post Hoc Analysis Of The Fuel Trial., David J. Goldberg, Victor Zak, Chenwei Hu, Adam M. Lubert, Rahul H. Rathod, Daniel J. Penny, Robert Whitehill, Kurt R. Schumacher, Salil Ginde, Michelle Ploutz, Gi Beom Kim, Todd T. Nowlen, Michael V. Dimaria, Benjamin P. Frischhertz, Jonathan B. Wagner, Kimberly E. Mchugh, Brian W. Mccrindle, Mark J. Cartoski, Jon Detterich, Anji T. Yetman, Anitha S. John, Marc E. Richmond, Matthew Files, R Mark Payne, Andrew S. Mackie, Christopher K. Davis, William B. Orr, Ruchira Garg, Kevin D. Hill, Jennifer S. Yauck, Michael G. Mcbride, James Yeager, Gail D. Pearson, Stephen M. Paridon, Bryan H. Goldstein

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: In the FUEL (Fontan Udenafil Exercise Longitudinal) trial, a positive treatment effect was identified for outcomes at the ventilatory anaerobic threshold but not for the primary outcome, oxygen consumption (Vo2 in participants with near-normal baseline exercise performance.

METHODS: Participants were divided into subgroups by baseline predicted peak Vo2 (< 80% versus ≥80%). Treatment effect was evaluated in those with a baseline peak Vo2 < 80% predicted and linear regression was performed to examine the interaction between subgroup and response to therapy for the primary and secondary outcomes.

RESULTS: Of the 379 participants with paired exercise data, 302 (80%) had a baseline peak Vo2 < 80% predicted. In this subgroup, the primary outcome of peak Vo2 improved significantly after 6 months of udenafil treatment, when compared with placebo (0.23±4.17 mL/kg per min versus -0.90±3.74 mL/kg per min; P=0.021). Secondary outcome measures, including Vo2 at …


Prenatal Diagnosis Of An Uncommon Form Of A Hypoplastic Left Heart Syndrome Variant., Anmol Goyal, Abigail Phillips, Kelsey Brattrud, Rita France, Maria Kiaffas Aug 2025

Prenatal Diagnosis Of An Uncommon Form Of A Hypoplastic Left Heart Syndrome Variant., Anmol Goyal, Abigail Phillips, Kelsey Brattrud, Rita France, Maria Kiaffas

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Mitral valve dysplasia syndrome (MVDS) is a rare cardiac defect requiring accurate prenatal identification and appropriate counseling, delivery planning, and postnatal management.

CASE SUMMARY: A 39-year-old woman underwent fetal echocardiography at 34 weeks' gestation owing to cardiomegaly and hydrops. Biatrial enlargement, mild to moderate atrioventricular valve insufficiency, dysplastic mitral valve apparatus, biventricular dilation with systolic dysfunction, endocardial fibroelastosis, hypoplastic aortic outflow, and severely restrictive atrial septum were attributed to MVDS. The plan for critical (class IV) delivery was modified with premature onset of labor at 36 weeks' gestation, leading to urgent cesarean section. Emergent balloon atrial septostomy was performed …


Daily Physical Activity And Cardiorespiratory Fitness In Adults With Down Syndrome With And Without Congenital Heart Disease., Julianne G. Clina, David A. White, Joseph R. Sherman, Jessica C. Danon, Daniel Forsha, Brian C. Helsel, Richard A. Washburn, Joseph E. Donnelly, Lauren T. Ptomey Jul 2025

Daily Physical Activity And Cardiorespiratory Fitness In Adults With Down Syndrome With And Without Congenital Heart Disease., Julianne G. Clina, David A. White, Joseph R. Sherman, Jessica C. Danon, Daniel Forsha, Brian C. Helsel, Richard A. Washburn, Joseph E. Donnelly, Lauren T. Ptomey

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Individuals with Down syndrome (DS) typically exhibit lower cardiorespiratory fitness and reduced moderate-to-vigorous physical activity (MVPA) compared to persons without disability. Approximately 50-55 % of individuals with DS have congenital heart disease (CHD), which is associated with cardiopulmonary deficiencies and reduced MVPA participation in non-DS populations. It is unknown if CHD related comorbidities compound with DS associated deficits in physical activity and fitness.

OBJECTIVE: To compare physical activity, cardiorespiratory fitness, and cardiovascular function, of persons with DS with and without CHD.

METHODS: Baseline data were used from a 12-month randomized controlled physical activity intervention of adults with DS. Participants …


Validity And Reliability Of The Tampa Scale For Kinesiophobia For Adolescents With Heart Disease., David A. White, William R. Black, Emily Cramer, Lindsey Malloy-Walton, Mollie Walton, Laura Martis, Brandy Enneking, Kelli M. Teson, Jessica S. Watson, Jami Gross-Toalson Jun 2025

Validity And Reliability Of The Tampa Scale For Kinesiophobia For Adolescents With Heart Disease., David A. White, William R. Black, Emily Cramer, Lindsey Malloy-Walton, Mollie Walton, Laura Martis, Brandy Enneking, Kelli M. Teson, Jessica S. Watson, Jami Gross-Toalson

Manuscripts, Articles, Book Chapters and Other Papers

PURPOSE: To assess the validity and reliability of a newly adapted Tampa Scale for KP for adolescents with HD (TSK-Heart-A).

METHODS: The TSK-Heart-A survey consists of 17 Likert scale items, producing a summary score (SS) ranging from 17 (low KP) to 68 (high KP). Following content and face validity, adolescents (age 12-18 yr) with arrhythmia disorders or Fontan palliation were recruited. The PROMIS pediatric anxiety and depression short forms, Pediatric Quality of Life Inventory (PedsQL) generic core and cardiac module, and a PA questionnaire for adolescents (PAQ-A) were used for criterion validity. The TSK-Heart-A was completed twice (20.4 ± 6.3 …


3- And 4-Dimensional Flow Cmr In D-Transposition Of The Great Arteries After Atrial Switch Operation., Sara Santiaguel, Laura Schoeneberg, Ugonna Nwankwo, Jorge Rosado Gonzalez, Wilson King May 2025

3- And 4-Dimensional Flow Cmr In D-Transposition Of The Great Arteries After Atrial Switch Operation., Sara Santiaguel, Laura Schoeneberg, Ugonna Nwankwo, Jorge Rosado Gonzalez, Wilson King

Manuscripts, Articles, Book Chapters and Other Papers

A 19-year-old man with D-transposition of the great arteries and pulmonary stenosis underwent atrial switch operation and pulmonary valvotomy in infancy. He was lost to follow-up and presented with multiple respiratory infections. An echocardiogram was inconclusive due to poor windows. Cardiac magnetic resonance (CMR) was performed using ferumoxytol 3-dimensional whole heart and 4-dimensional flow pulse sequences, which demonstrated this patient's complex anatomy and physiology. CMR revealed severe pulmonary venous baffle obstruction. A 3-dimensional printed model was created from the CMR and provided key information for planning the complex catheterization approach required to stent the pulmonary venous baffle.


Effects Of Genetic Polymorphisms On The Oct1 And Oct2-Mediated Uptake Of Atenolol, Mollie Margaret Walton Md, Jonathan Wagner Do, Ceclia E. Villanueva Phd, Bruno Hagenbuch Phd May 2025

Effects Of Genetic Polymorphisms On The Oct1 And Oct2-Mediated Uptake Of Atenolol, Mollie Margaret Walton Md, Jonathan Wagner Do, Ceclia E. Villanueva Phd, Bruno Hagenbuch Phd

Research Days

This project characterizes the role of OCT1 and OCT2 on cellular uptake of atenolol and tests the hypothesis that OCT1 and OCT2 proteins with genetic polymorphisms, when expressed in vitro, will result in altered cellular uptake of atenolol relative to the reference genotype. This approach utilizes transiently transfected OCT1 and OCT2 cell assays to perform time-dependent atenolol uptake experiments to generate kinetics information. The goal is to provide a better understanding of polymorphisms associated with OCT1 and OCT2 expression and function as a means to individualize pharmacologic therapy for atenolol.


Duration Of Labor And Umbilical Artery Acidosis In Critical Congenital Heart Disease, Sarah Gwazdacz Md, Teresa Nguyen Bs, Mary Donofrio Md, Faap, Facc, Fase, Timothy Bennett Md, Amanda Mcintosh Md, Faap, Facc. May 2025

Duration Of Labor And Umbilical Artery Acidosis In Critical Congenital Heart Disease, Sarah Gwazdacz Md, Teresa Nguyen Bs, Mary Donofrio Md, Faap, Facc, Fase, Timothy Bennett Md, Amanda Mcintosh Md, Faap, Facc.

Research Days

Standard delivery protocols for most prenatally diagnosed CCHD is induction of labor at 39 weeks with aims of a vaginal delivery (VD) near a tertiary care center. There is no benefit to planned Cesarean delivery (CD) over VD for most infants with CCHD. However, longer durations of labor (DOL) in CCHD have been described as a risk factor for umbilical artery (UA) acidosis. We aim to describe the relationship between DOL and UA pH and neonatal outcomes in those with prenatally diagnosed CCHD.

Retrospective chart review of prenatally diagnosed with CCHD born at Children’s Mercy from 2012 – 2023. Inclusion …


Variables Affecting Neurodevelopmental Outcome In Infants With Critical Congenital Heart Disease, Elizabeth Loughman, Julie Weiner Do, Marcie Files Md May 2025

Variables Affecting Neurodevelopmental Outcome In Infants With Critical Congenital Heart Disease, Elizabeth Loughman, Julie Weiner Do, Marcie Files Md

Research Days

Background: The mortality of cardiac conditions in infancy is improving with advances in surgical planning and medical management. Neurologic morbidity remains significant. Clinically correlating an infant with their neurologic exam, MRI/EEG, and subsequent developmental testing remains a challenge. Counseling regarding neurologic outcomes after cardiac intervention is difficult due to the wide range of findings that can sometimes be clinically irrelevant.

Purpose: The goal of this study is to identify factors associated with poor neurodevelopmental outcomes in infants with critical congenital heart defects (CCHD) as assessed by the Bayley Scales of Infant and Toddler Development Edition IV (Bayley).

Methods: This is …


Ecmo Without Compressions Versus Ecpr For Lcos Following Cardiac Surgery, Amjad Halloum, Apurva Panchal, Sarah Tabbutt, Martina Steurer, Mousumi Banerjee, John Charpie, Javi Lasa, Amy Mccammond, Katie Moynihan, Nancy Ghanayem, Tia Raymond May 2025

Ecmo Without Compressions Versus Ecpr For Lcos Following Cardiac Surgery, Amjad Halloum, Apurva Panchal, Sarah Tabbutt, Martina Steurer, Mousumi Banerjee, John Charpie, Javi Lasa, Amy Mccammond, Katie Moynihan, Nancy Ghanayem, Tia Raymond

Research Days

A retrospective study looking into ECMO vs eCPR outcomes in the CICU. By refining the approach to postoperative ECMO initiation, this study aims to contribute valuable insights that could improve survival and reduce complications in pediatric cardiac surgery patients.


Assessing Ecg Abnormalities In Pediatric Athletes: A Retrospective Study On Incidence And Implications For Screening Protocols, Arkadeep Ghosh, Mark Gelatt, Lindsey Malloy-Walton, Ashley K. Sherman May 2025

Assessing Ecg Abnormalities In Pediatric Athletes: A Retrospective Study On Incidence And Implications For Screening Protocols, Arkadeep Ghosh, Mark Gelatt, Lindsey Malloy-Walton, Ashley K. Sherman

Research Days

Background: Utilization of Electrocardiogram (ECG) screening in pediatric athletes remains controversial. The American Heart Association (AHA) hasn't supported the use of mandatory ECG screening for pediatric sports evaluations. The financial burden universal ECG screening in pediatric athletes poses on the healthcare system has supported that screening may not be justified given the low incidence of significant cardiac disease.

Objectives/Goal: This study aimed to examine the incidence, type of ECG abnormalities, and associated risk factors in healthy pediatric athletes.

Methods/Design: A single center retrospective cohort study was conducted on a local male youth competitive soccer program, ages 11-18. Exempt status was …


Spectrum Of Coronary Artery Involvement With Multisystem Inflammatory Syndrome In Children Versus Kawasaki Disease., Simon Lee, Ashraf S. Harahsheh, Geetha Raghuveer, Michael A. Portman, Arash A. Sabati, Michael Khoury, Nagib Dahdah, Marianna Fabi, Supriya S. Jain, Audrey Dionne, Kyle Runeckles, Frederic Dallaire, Nadine F. Choueiter, Tyler H. Harris, Matthew D. Elias, Anji T. Yetman, Balasubramanian Sundaram, Luis M. Garrido-Garcia, Nilanjana Misra, Cedric Manlhiot, Pedrom Farid, Brian W. Mccrindle May 2025

Spectrum Of Coronary Artery Involvement With Multisystem Inflammatory Syndrome In Children Versus Kawasaki Disease., Simon Lee, Ashraf S. Harahsheh, Geetha Raghuveer, Michael A. Portman, Arash A. Sabati, Michael Khoury, Nagib Dahdah, Marianna Fabi, Supriya S. Jain, Audrey Dionne, Kyle Runeckles, Frederic Dallaire, Nadine F. Choueiter, Tyler H. Harris, Matthew D. Elias, Anji T. Yetman, Balasubramanian Sundaram, Luis M. Garrido-Garcia, Nilanjana Misra, Cedric Manlhiot, Pedrom Farid, Brian W. Mccrindle

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: There is significant overlap in clinical features between multisystem inflammatory syndrome in children (MIS-C) and Kawasaki disease (KD). We sought to compare the prevalence, severity, and associated factors for coronary artery (CA) involvement.

METHODS AND RESULTS: From January 1, 2020 through January 31, 2023, 1191 patients with MIS-C and 554 patients contemporaneously diagnosed with KD were enrolled into the International Kawasaki Disease Registry. Demographic and clinical features, laboratory values, maximum Z score in any CA branch +2.5 or greater) were determined separately for each diagnosis using multivariable logistic regression analyses. The prevalence of CA aneurysms was lower for MIS-C …