Cardiology Commons^™

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

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

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

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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

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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

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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

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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 …

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

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• 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

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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

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

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

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

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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 …

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

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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

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

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

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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

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

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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

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

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

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.

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 …

Factors Associated With Shock At Presentation In Kawasaki Disease Versus Multisystem Inflammatory Syndrome In Children Associated With Covid-19., Supriya S. Jain, Ashraf S. Harahsheh, Simon Lee, Geetha Raghuveer, Nagib Dahdah, Michael Khoury, Michael A. Portman, Melissa Wehrmann, Arash A. Sabati, Marianna Fabi, Deepika Thacker, Nilanjana Misra, Mark D. Hicar, Nadine F. Choueiter, Matthew D. Elias, Audrey Dionne, William B. Orr, Jacqueline R. Szmuszkovicz, Seda Selamet Tierney, Luis Martin Garrido-Garcia, Frederic Dallaire, Balasubramanian Sundaram, Deepa Prasad, Tyler H. Harris, Elizabeth Braunlin, Elisa Fernandez Cooke, Cedric Manlhiot, Pedrom Farid, Brian W. Mccrindle, International Kawasaki Disease Registry

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: While clinical overlap between Kawasaki disease (KD) and multisystem inflammatory syndrome in children (MIS-C) has been evident, information regarding those presenting with shock has been limited. We sought to determine associations with shock within and between diagnosis groups.

METHODS: The International KD Registry enrolled contemporaneous patients with either KD or MIS-C from 39 sites in 7 countries from January 1, 2020, to January 1, 2023. Demographics, clinical features and presentation, management, laboratory values, and outcomes were compared between the diagnosis and shock groups.

RESULTS: Shock at presentation was noted for 19 of 672 KD patients (2.8%) and 653 of …

Two-Dimensional Speckle Tracking Of The Ascending Aorta: A Novel Approach To Evaluate Arterial Stiffness In Pediatric Patients With Repaired Conotruncal Anomalies Using Echocardiography., Anmol Goyal, Anitha Parthiban, David A. White, Daniel Forsha, Doaa Aly

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Children with repaired conotruncal abnormalities (CTA) are at risk of progressive aortic dilation and deteriorating aortic elasticity even after surgical correction. Strain imaging, using a 2-dimensional speckle tracking echocardiography (2D-STE), has been used for arterial stiffness assessment, but pediatric data are still lacking. We investigated the feasibility, reproducibility, and clinical value of 2D-STE derived ascending aorta (AAo) stiffness in children with repaired CTA.

METHODS: 22 pediatric patients with repaired CTA were included along with 25 age- and sex- matched healthy controls (mean age 10.2 ± 4.5 years). Conventional 2D echocardiographic and 2D-STE assessment of AAo mechanics was performed. M-mode …

Analyzing The Creation And Use Of Abbreviations In Cardiology And Cardiac Imaging Society Guidelines., Christopher R. Green, Rui Zhang, Raymond F. Stainback, Sofia W. Ye, Daniel Forsha, Enrique Garcia-Sayan, Jeffrey C. Hill, Carol Mitchell, Vera H. Rigolin, Vandana Sachdev, Partho P. Sengupta, Vincent L. Sorrell, Jordan B. Strom, Alexander M. Ye, Cynthia C. Taub

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Abbreviation use in clinical and academic cardiology is widespread, yet there are few guidelines regulating the creation and utilization of abbreviations. Inconsistent abbreviations can introduce ambiguity and pose challenges to practice and research.

OBJECTIVES: The authors aimed to analyze how abbreviations are created and utilized in general cardiology and cardiac imaging society guidelines in order to assess whether ambiguities and discrepancies exist between societies.

METHODS: Abbreviation data were collected from 7 national and international societies of general cardiology and cardiac imaging over a 6-year span (2018-2023). Data were linguistically coded for abbreviation type, unique occurrence, meaning or sense count, …

Diagnostic Accuracy Prior To Congenital Heart Defect Surgery: A Multicenter Collaboration., Shobha S. Natarajan, Kasey Chaszczewski, Chris Penney, Steve Ampah, Douglas Ryba, Andrea T. Kennedy, Robert Olsen, Shubhika Srivastava, Matthew J. Campbell, Mya Carney, Carol Prospero, Lisa Elliott, Carlie Brewer, Michael Dimaria, Nitin Madan, Seda Tierney, Meaghan Beattie, Ritu Sachdeva, Joan Lipinski, Kenan W D Stern, Grace Kong, Preeta Dhanantwari, Elena N. Kwon, Hari Rajagopal, Carolyn Taylor, Tammy Churchill, Aura A. Sanchez Mejia, Deidra Abenlah Ansah, Anitha Parthiban, Hasti Sanandajifar, Sowmya Balasubramanian, David A. Parra, Kimberly Crum, Corey Stiver, Aarti H. Bhat, Pei-Ni Jone, Stefani Samples, Kathleen Van't Hof, Curt Degroff, Dalia Lopez-Colon, Meryl S. Cohen

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Echocardiography is the mainstay for diagnosing congenital heart disease (CHD). Diagnostic errors can lead to suboptimal surgical outcomes.

OBJECTIVES: This multicenter pediatric echocardiography collaborative learning initiative explores reasons for diagnostic errors, investigates associations between patient- and center-specific factors and errors, and relays the benefits of a multicenter approach to decrease these errors as a first step to improve CHD surgical outcomes.

METHODS: Participating centers submitted diagnostic evaluations on patients prior to 2-ventricle repair into a central database. We held virtual meetings to revise variables and discuss cases to learn from each other.

RESULTS: Fourteen pediatric echocardiography laboratories entered data …

Tetralogy Of Fallot: Anatomy, Physiology, And Outcomes, Edo Bedzra, Eli Contorno, Herra Javed, Amna Qasim, James St Louis, Taufiek Konrad Rajab

Manuscripts, Articles, Book Chapters and Other Papers

Since the first identification of Tetralogy of Fallot in 1671, consisting of a combination of anatomical defects including biventricular origin of the aorta, maligned ventricular septal defect, overriding aorta, and narrowing or atresia of the pulmonary outflow tract. The first successful operation consisted of a shunt between the left subclavian artery and pulmonary artery. Following this palliative procedure, complete repair is performed once the patient reaches indicative criteria. Since the first attempts at surgical palliation and repair, techniques and outcomes have improved drastically. Definitive repair of Tetralogy of Fallot consists of a multi-patch closure of any Ventricular Septal Defect along …

Case Report: Prominent Coronary Artery Flow In Fetuses With Congenital Heart Disease, Is It A Marker Of In Utero Distress?, Mohamed Aashiq Abdul Ghayum, Maria Kiaffas, Ashley Warta, Melanie Kathol, David Christopher Mundy, Kelsey Brattrud, Nitin Madan

Manuscripts, Articles, Book Chapters and Other Papers

Prominent coronary artery (CA) flow observed on a fetal echocardiogram has been associated with fetal growth restriction and myocardial dysfunction. We present two cases with this finding, in the presence of congenital heart disease (CHD) and absence of growth restriction or myocardial dysfunction. Both the cases rapidly progressed to extremis, necessitating emergent delivery. Our cases highlight the importance of recognizing prominent CA flow in fetuses with CHD as a potential marker for in utero distress.

The Norwood With Blalock-Taussig-Thomas Shunt—Worth A Second Look?, Manasa Gadiraju, Dhaval Chauhan, Alexandra Schray, James O'Brien, Edo Bedzra

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Background: Shunt repair for the Norwood procedure with either the right ventricle to pulmonary artery shunt (RVPAS) or the modified Blalock-Taussig-Thomas Shunt (BTTS) varies by institution and surgeon preference. Shunt choice has been informed by landmark trials including the Single Ventricle Reconstruction trial and modern outcomes data may engender future complementary studies. Methods: We conducted a retrospective analysis of all patients who underwent the Norwood procedure from 2014–2022 at a single center to compare outcomes by shunt type. The primary outcome measure was freedom from death or transplant. Secondary outcome measures included hospital length of stay, complications, and unplanned interventions. …