Medicine and Health Sciences Commons^™

Gender Dysphoria In Adolescents With Ehlers-Danlos Syndrome., Jordan T. Jones, William R. Black, Christine Moser, Eric T. Rush, Lindsey Malloy-Walton

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVES: Ehlers-Danlos Syndrome represents a family of heritable connective tissue disorders that include joint hypermobility, tissue fragility, and skin hyperextensibility. Ehlers-Danlos Syndrome presents with clinical sequela across multiple body systems that require multidisciplinary care. Little is known about adolescents with Ehlers-Danlos Syndrome who are transgender and gender diverse. To date, there have been no reports of transgender and gender diverse youth in pediatric patients with Ehlers-Danlos Syndrome. The objective of this study was to characterize transgender and gender diverse adolescents with Ehlers-Danlos Syndrome seen in a pediatric multidisciplinary specialty clinic.

METHODS: A retrospective chart review was performed and it was …

Cardiac Biomarkers In Differentiating Kawasaki Disease And Multisystem Inflammatory Syndrome In Children Associated With Covid-19, Mollie Walton, Geetha Raghuveer, K Kamakoti, N Dahdah, L Garrido, S Tierney, T Harris, M Khoury, M Hicar, E Braunlin, D Thacker, M Khare, F Dallaire, R Lowndes, I Glassmeyer, J Ballweg, G Goldenberg, S Merves, C Manlhiot, P Farid, Bw Mccrindle

Posters

Introduction – Kawasaki disease (KD) and Multisystem Inflammatory Syndrome in Children (MIS-C) associated with COVID-19 show considerable clinical overlap making differentiation challenging, particularly in the absence of evidence of evidence of prior COVID-19. Hypothesis – Cardiac biomarkers can differentiate KD from MIS-C. Methods – The International KD Registry enrolled n=2903 contemporaneous KD, MIS-C and acute COVID-19 pediatric patients from 42 sites in 8 countries from January 2020 through July 2022. The study population was confined to 1489 MIS-C patients meeting CDC criteria with confirmed evidence of prior COVID-19 infection and 387 KD patients meeting AHA guideline criteria with confirmed evidence …

Microrna Expression Levels Change In Neonatal Patients During And After Exposure To Cardiopulmonary Bypass., Lance Hsieh, Lan N. Tu, Alison Paquette, Quanhu Sheng, Shilin Zhao, Douglas C. Bittel, James O'Brien, Kasey Vickers, Peter Pastuszko, Vishal Nigam

Manuscripts, Articles, Book Chapters and Other Papers

Background The systemic inflammation that occurs after exposure to cardiopulmonary bypass (CPB), which is especially severe in neonatal patients, is associated with poorer outcomes and is not well understood. In order to gain deeper insight into how exposure to bypass activates inflammatory responses in circulating leukocytes, we studied changes in microRNA (miRNA) expression during and after exposure to bypass. miRNAs are small noncoding RNAs that have important roles in modulating protein levels and function of cells. Methods and Results We performed miRNA-sequencing on leukocytes isolated from neonatal patients with CPB (n=5) at 7 time points during the process of CPB, …

Safety, Timing, And Outcomes Of Early Postoperative Cardiac Catheterization Following Congenital Heart Surgery, Karoline Krzywda, Jeremy Affolter, Darcie Al-Hassan, William Gibson, Ryan Romans, Kelly S. Tieves

Posters

Intro: Historically, early postoperative (<6weeks) cardiac catheterization (EPOCC) was considered high risk and often delayed. Recently, the safety of early postoperative cardiac catheterization has been demonstrated. The timing of catheterization in the struggling postoperative patient remains variable and optimal timing remains uncertain. There is limited data on the impact of EPOCC on outcomes such as length of stay, duration of mechanical ventilatory or extracorporeal support, and surgical or catheter- based interventions. The aim of this study was to describe our experience with EPOCC and its impact on management, length of stay, and duration of mechanical ventilatory support. Methods: This is a descriptive retrospective cohort study of patients who underwent cardiac surgery between 01/01/2010 and 12/31/2019 and cardiac catheterization within 30 days after surgery. Patients with catheterization for LA decompression on ECMO, endomyocardial biopsy, or BAS post hybrid procedure were excluded. We collected information surrounding timing, indication, and complications of catheterization, duration of mechanical ventilation/ECMO, and ICU/hospital LOS and compared these differences in patients who underwent EPOCC < 72hours (earlier) versus >72hours (later). Data was analyzed using IBM SPSS, version 28. Chi-square and Student’s t-test, or appropriate nonparametric tests were used to compare categorical and continuous variables, respectively. Results: There were 2542 surgeries performed during the study period with 167 patients undergoing cardiac catheterization within the defined 30 days. Twenty-six patients met exclusion criteria, leaving 141 patients for analyses. Median time from surgery to EPOCC was 10 days (1-30), with 26 patients(18.4%) having an earlier EPOCC. Interventions were performed in 70 patients (49.6%), not statistically different in those with earlier vs later EPOCC [n=9 (12.9%) vs …

Impact Of Remote Monitoring During The Interstage Period On Outcomes In Single Ventricle Patients Across Socioeconomic Groups, Bianca Cherestal, Lori Erickson, Janelle R. Noel-Macdonnell, Girish S. Shirali, Hayley S. Hancock, Doaa Aly, Natalie Jayaram

Posters

Background: Despite improvements in outcomes over time, morbidity and mortality for infants with single ventricle (SV) heart disease remains high. Among other risk factors for mortality, infants of low socioeconomic status (SES) are known to be particularly vulnerable following stage 1 palliation. We sought to determine whether use of a novel remote monitoring program, CHAMP (Cardiac High Acuity Monitoring Program), mitigates differences in outcomes by SES for infants with SV during the interstage period. Methods: Using the CHAMP database, we identified 607infants across 11 institutions enrolled in the program between 2014–2021. All enrolled patients’ families were provided with a Windows-based …

Association Of Acute Anti-Inflammatory Treatment With Medium-Term Outcomes For Coronary Artery Aneurysms In Kawasaki Disease., Kevin G. Friedman, Brian W. Mccrindle, Kyle Runeckles, Nagib Dahdah, Ashraf S. Harahsheh, Michael Khoury, Sean Lang, Cedric Manlhiot, Adriana H. Tremoulet, Geetha Raghuveer, Elif Seda Selamet Tierney, Pei-Ni Jone, Jennifer S. Li, Jacqueline R. Szmuszkovicz, Kambiz Norozi, Supriya S. Jain, Angela T. Yetman, Jane W. Newburger, International Kawasaki Disease Registry

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: The impact of adjunctive anti-inflammatory treatment on outcomes for patients with Kawasaki disease (KD) and coronary artery aneurysms (CAAs) is unknown.

METHODS: Using data from the International KD Registry in patients with ≥ medium CAA we evaluate associations of treatment with outcomes and major adverse cardiac events (MACE).

RESULTS: Medium or large CAA was present in 527 (32%) patients. All were treated with intravenous immunoglobulin (IVIG), 70% were male, and the median age was 1.3 years (interquartile range: 0.4-4.0 years). The most common acute therapies included single IVIG alone in 243 (46%), multiple IVIG in 100 (19%), multiple IVIG …

Myocardial Work By Strain Echocardiography: A Predictor Of Exercise Capacity In The Fontan Population, Amulya Buddhavarapu, Suma Goudar, David A. White, Daniel Forsha, Doaa Aly

Posters

INTRODUCTION: Global myocardial work index (GWI) obtained by non-invasive pressure-strain loops is a fast-emerging tool to assess myocardial contractility, independent of loading conditions. Data about the feasibility and utility of this modality in single ventricles are still lacking. AIM: We aimed to explore the relationship between the non-invasive myocardial work indices and exercise parameters in the Fontan population. METHOD: Fontan patients were prospectively evaluated by exercise testing and echocardiography. The systemic ventricle was tracked in 3 orthogonal apical planes for global longitudinal strain (GLS). Pressure-strain loops were generated against brachial blood pressure (surrogate for ventricular systolic pressure in the absence …

Safety, Timing And Outcomes Of Early Postoperative Cardiac Catheterization Following Congenital Heart Surgery, Karoline Krzywda

Research Days

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Background: Early postoperative ( < 6weeks) cardiac catheterization (EPOCC) has been considered high risk and often delayed. Recently, the safety of EPOCC has been demonstrated. The optimal timing of catheterization and its outcomes in struggling postoperative patients remains uncertain.

Objective: To describe EPOCC in our center and its impact on length of stay (LOS), number of mechanical ventilation days, number of ECMO days, and catheter-based interventions.

Methods: This is a retrospective cohort study of patients who underwent cardiac surgery between 01/01/2010 and 12/31/2019 and EPOCC within 30 days after surgery. Cohorts were early EPOCC (≤72 hr) vs late EPOCC ( >72 hr). Data on timing, indication, and complications of EPOCC; days of mechanical ventilation and ECMO; and CICU/total hospital LOS were collected. Data was analyzed using chi-square and Student’s t-test, …

Ekg Technology: It Is Time For A Change!, Megan Carroll

Research Days

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Background: Technology for obtaining pediatric EKGs has not changed in over 70 years. Current guidelines recommend a 12-lead EKG, which takes approximately 7 minutes to complete. Newer adult technology utilizes between 1-6 leads for EKG analysis. There has not been a study in pediatrics to assess the minimum number of leads required to attain diagnostic accuracy.

Objectives/Goal: To determine the appropriate number of leads needed to read pediatric EKGs safely and accurately.

Methods/Design: This is a single center systematic review that included randomly selected EKGs from our hospital database (2017-2020) read by two independent pediatric …

Neonatal Hypoxia Due To Pulmonary Artery Thrombus, Bianca Cherestal

Research Days

Background: Pulmonary artery thrombus is rare and should be considered in neonates with refractory hypoxia.

Case: Preterm female neonate had persistent hypoxia after birth despite intubation, high-frequency oscillatory ventilation (FiO2 100%), and inhaled nitric oxide. Delivery was via c-section at 33 weeks 4 days for new onset hydrops and poor biophysical profile. Echocardiogram revealed echo brightness in the lumen of the left pulmonary artery (LPA) with no color flow (figure 1a), concerning for occlusive thrombus. Chest CT angiogram confirmed the diagnosis (figure 1b, c). Hematology was consulted and recommended systemic thrombolysis (tissue plasminogen activator, tPA) with concomitant anticoagulation (Bivalirudin). Imaging …

Iv Sotalol Use In Pediatric And Congenital Heart Patients: A Multicenter Registry Study., Lindsey Malloy-Walton, Nicholas H. Von Bergen, Seshadri Balaji, Peter S. Fischbach, Jason M. Garnreiter, S Yukiko Asaki, Jeffrey P. Moak, Luis A. Ochoa, Philip M. Chang, Hoang H. Nguyen, Akash R. Patel, Christa Kirk, Ashley K. Sherman, Jennifer N. Avari Silva, J Philip Saul

Manuscripts, Articles, Book Chapters and Other Papers

Background There is limited information regarding the clinical use and effectiveness of IV sotalol in pediatric patients and patients with congenital heart disease, including those with severe myocardial dysfunction. A multicenter registry study was designed to evaluate the safety, efficacy, and dosing of IV sotalol. Methods and Results A total of 85 patients (age 1 day-36 years) received IV sotalol, of whom 45 (53%) had additional congenital cardiac diagnoses and 4 (5%) were greater than 18 years of age. In 79 patients (93%), IV sotalol was used to treat supraventricular tachycardia and 4 (5%) received it to treat ventricular arrhythmias. …

Comprehensive Evaluation Of Left Ventricular Deformation Using Speckle Tracking Echocardiography In Normal Children: Comparison Of Three-Dimensional And Two-Dimensional Approaches., Doaa Aly, Nitin Madan, Laura Kuzava, Alison Samrany, Anitha Parthiban

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Three-dimensional (3D) speckle tracking echocardiography (STE) can overcome some of the inherent limitations of two-dimensional (2D) STE; however, clinical experience is lacking. We aimed to assess and compare the feasibility, agreement, and reproducibility of left ventricular (LV) global longitudinal (GLS), and regional strain by 3D vs 2D STE in normal children.

METHODS: Healthy pediatric subjects (n = 105, age mean = 11.2 ± 5.5 years) were prospectively enrolled. Three-dimensional and 2D LV GLS, as well as regional strain in 16 myocardial segments were quantified. Bland Altman analysis, intra- class correlation coefficients (ICC), percent error and linear regression were used …

Surgical Management Of Atrial-Esophageal Fistula As A Complication Of Atrial Fibrillation Ablation., Amber Duda, Kevin Beers, Mohi Mitiek

Manuscripts, Articles, Book Chapters and Other Papers

Atrial-esophageal fistula (AEF) is a rare, but life-threatening complication of ablative treatments for atrial fibrillation. Although the incidence of this complication is low, the mortality is very high. There are many surgical approaches to this disease but we offer a novel technique to reduce the number of incisions used and provides central cannulation. It also allows for repair of both the esophagus and atrium and buttresses these repairs, which have both been shown to decrease morbidity and mortality. The technique has been successful in our three patients and can be considered as an approach to surgical management of AEF.