Medicine and Health Sciences Commons^™

Double Aortic Arch With Atresia Of The Left Aortic Arch Proximal To The Left Common Carotid Artery In A Patient With Phace Syndrome – A Management Conundrum, Mohamed Aashiq Abdul Ghayum, Anmol Goyal, Aliessa P. Barnes, Sanket Shah

Research Days

Background Cardiac anomalies in PHACE syndrome (P: posterior fossa anomalies, H: hemangiomas, A: arterial lesions, C: cardiac abnormalities, E: abnormalities of the eye) often consists of complex aortic arch anomalies with double aortic arch (DAA) being an uncommon association. Atresia of the proximal segment of the left arch in DAA is extremely rare and can pose a diagnostic and management conundrum due to formation of a loose vascular ring and asymptomatic nature as seen in our case.

Case Description A 5-year-old male, diagnosed with PHACE syndrome, at a different center was noted to have an incidental finding on head imaging …

Rare Complications Associated With Use Of Bovine Pericardium, Mohamed Aashiq Abdul Ghayum, Nitin Madan, William J. Gibson, Sanket Shah

Research Days

Background

Bovine Pericardium (BP), with numerous and improving preparation techniques, has been used extensively for over 50 years in repair of simple and complex congenital heart defects. It has several advantages over other surgical materials. However, there is a paucity of data in the pediatric population regarding complications associated with BP. Herein, we report two rare complications noted with BP use.

Case Description

Case 1. A 14-month-old male, with trisomy 21 and complete Atrioventricular canal defect, repaired using BP. A routine surveillance echocardiogram showed a mass attached to the atrial septum and atrial aspect of the left atrioventricular valve (Figure …

Cardiac Biomarkers Differentiate Kawasaki Disease From Multisystem Inflammatory Syndrome In Children Associated With Covid-19, Mollie Walton

Research Days

Background: Kawasaki disease (KD) and Multisystem Inflammatory Syndrome in Children (MIS-C) associated with COVID-19 show considerable clinical overlap. The goal of this study was to determine if cardiac biomarkers can differentiate KD from MIS-C.

Objectives/Goal: The goal of this study was to determine if cardiac biomarkers differentiate KD from MIS-C.

Methods/Design: The International Kawasaki Disease Registry enrolled (n=2903) contemporaneous KD and MIS-C patients1st, 2020, through June 30th, 2022. The study population was confined to KD patients meeting American Heart Association KD criteria with no prior COVID-19 infection and MIS-C patients meeting Centers for Disease Control and Prevention criteria with confirmed …

Association Between Remote Monitoring And Interstage Morbidity And Mortality In Single Ventricle Patients Across Socioeconomic Groups, Bianca Cherestal

Research Days

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.

Objectives/Goal: 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/Design: Using the CHAMP database, we identified 610 infants across 11 institutions enrolled in the program between 2014–2021. All enrolled patients’ families were provided with …

Therapeutic Plasma Exchange To Alleviate Ventricular Tachycardia After Diphenhydramine Ingestion, Kaushal Dosani, Lejla Music Aplenc, Stephen Pfeiffer

Research Days

Background: The Covid-19 pandemic has adversely affected the mental health of children causing an increased incidence of suicide attempts. Diphenhydramine is a common household medication and is frequently ingested by children. Toxic doses of diphenhydramine can affect the cardiovascular and central nervous system. In the heart, diphenhydramine blocks fast sodium channels and potassium channels which can result in conduction abnormalities including sinus tachycardia, widening of QRS duration, ventricular tachycardia and torsades de pointes. Massive ingestion can cause severe cardiovascular collapse which may require ECMO support

Objectives/Goal: To describe a novel method of clearing highly protein-bound diphenhydramine molecules from circulation after …

Improving Imaging Practices Following Arterial Switch Operation, Mollie Walton, Nir Atlas, Sanket Shah

Research Days

Problem Statement/Question: Long-term risk in post-operative cardiac patients exists, and morbidity may be avoided with advanced imaging surveillance.

Background/Project Intent (Aim Statement): The Appropriate Use Criteria (AUC) for Multimodality Imaging During Follow-Up Care of Patients with Congenital Heart Disease (CHD) was published in 2020 by the American College of Cardiology Foundation. This includes a subsection for D-Looped Transposition of the Great Arteries (D-Loop TGA). The arterial switch operation (ASO) is the preferred method for surgical repair of D-Loop TGA. Appropriate and timely use of advanced imaging is essential in optimizing long-term postsurgical outcomes. The objective of this quality improvement (QI) …

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 …

Neonatal Arch Obstruction: Not Always A Coarctation, Karoline Krzywda

Research Days

Background: Spontaneous neonatal arch thrombosis is a rare, highly lethal cause of aortic arch obstruction.

Case: A 1-day-old male infant presented failing CCHD screen, on prostaglandin infusion for echocardiographic concerns of aortic coarctation. Repeat echocardiogram revealed severe arch obstruction due to a large, 7.5x5mm echogenic mass, at the aortic isthmus (Fig1A) with right to left systolic PDA flow. CT angiography confirmed presence of a large aortic arch thrombus (Fig1B). Bivalirudin infusion was initiated without change in thrombus size after 2 days. Tissue plasminogen activator (tPA) was subsequently administered with significant reduction in thrombus size to 3x4mm (Fig1C) on day 1 …

Atrial Standstill In A Pediatric Patient With Scn5a Mutation Following Procainamide Challenge, Anmol Goyal

Research Days

Background: Atrial standstill (AS) is a rare arrhythmia characterized by absence of electrical and mechanical atrial activity associated with SCN5A channelopathy.

Case: An 18 year old male with structurally normal heart, frequent sinus pauses, nonsustained atrial tachycardia and high-grade block was found to have SCN5A mutation c.3823G>A (p.Asp1275Asn). An electrophysiology study (EPS) with high density voltage mapping of the right atrium was done (Fig 1a). Nonsustained multifocal atrial tachycardia was induced without ablative targets (Fib 1b). Procainamide challenge was negative for Brugada, however induced AS (Fig 1c-d). No atrial capture could be achieved at maximal output. Empiric atrial lead …

Constrictive Pericarditis After Repair Of A Ruptured Sinus Of Valsalva, Sarah Studyvin, Laura Kuzava

Research Days

Background: Constrictive pericarditis is an uncommon complication of cardiac surgery. We report a patient who developed constrictive pericarditis after ruptured sinus of Valsalva (RSOV) repair.

Methods: A 23-year-old male presented with exertional dyspnea one year after RSOV repair. TTE showed a small, circumferential effusion with thickened pericardium, ventricular septal bounce, left atrial enlargement, diastolic hepatic flow reversal, and trivial mitral regurgitation without stenosis. He underwent cardiac catheterization, which revealed elevated filling pressures (RVEDP 16 mmHg, LVEDP 18 mmHg), RVEDP/RVSP ratio < 0.5, and a low cardiac index (1.65 L/min/m2). Cardiac MRI confirmed pericardial thickening with paradoxic septal motion, dilated pulmonary veins and retrograde flow in the SVC.

Results: Pericardiectomy of thickened and adherent pericardium was performed. The central venous pressure decreased from 23 to 7 mmHg and …

Prenatal Diagnosis Of An Uncommon Form Of A Hypoplastic Left Heart Syndrome Variant., Anmol Goyal

Research Days

Background: Mitral valve dysplasia syndrome (MVDS) is a rare form of congenital heart disease, similar to hypoplastic left heart syndrome (HLHS). Prenatal identification is important for counseling, delivery planning and postnatal management.

Case: A 39 year-old woman underwent fetal echocardiography at ~34 weeks gestation for evaluation of fetal cardiomegaly and hydrops revealing: biatrial enlargement, mild-to-moderate mitral and tricuspid valve insufficiency, echobright mitral valve apparatus, biventricular dilation, severe LV fibroelastosis (EFE) and systolic dysfunction, mild hypoplasia of aortic valve annulus and aortic arch, and a thick restrictive atrial septum (RAS) with left to right flow (Fig 1a-c).

Decision‐making: Although critical aortic …

Online Echocardiography Modules For Pediatric Cardiology Fellows, Sarah Studyvin, Doaa Aly, Tyler Johnson, Laura Kuzava, Alison Samrany, Nitin Madan, Sanket Shah

Research Days

Background: Adequate echocardiographic (echo) education is integral to successful cardiology training. Online education is proven to be effective in other specialties, but pediatric echo training data are lacking.

Objectives/Goal: Our aim was to design and assess the role of a novel online module-based curriculum to enhance pediatric cardiology fellows’ echo education.

Methods/Design: Four interactive modules, focused on principles and interpretation of a normal echo, were delivered to fellows. Content included high quality 2D and 3D echo clips as well as interactive quizzes and could be accessed from a phone, tablet, or computer. All fellows completed pre- and post-tests, validated by …

Pediatric Pulmonary Artery Aneurysms Causing Pulmonary Hemorrhage: A Case Of Transcatheter Intervention While On Va Ecmo Support, Igor Areinamo

Research Days

Background: The incidence of pulmonary artery aneurysm (PAA) in children remains unknown. There are few reports of a transcutaneous interventional approach to address this type of pathology. Management of PAAs while on VA ECMO has not been described in pediatrics.

Objectives/Goal:

Methods/Design:

Case: 9-year-old previously healthy male presented with MRSA associated ARDS and sepsis requiring urgent VA ECMO cannulation. Chest CT done on ECMO day 22 showed development of right sided PAAs. Patient had an episode of significant pulmonary hemorrhage. Angiogram demonstrated two fusiform right PAAs, one of which had ruptured and was bleeding into the lung parenchyma. Patient had …

Standardized Point-Of-Care Ultrasound (Pocus) Set-Up For Cardiac Evaluation During Covid-19 Pandemic In A Pediatric Population., Anmol Goyal

Research Days

Background: The novel coronavirus SARS-CoV2 is known to cause significant cardiac dysfunction, effusion, thromboembolism and coronary dilation. Currently no pediatric specific guidelines exist from ACC or ASE for use of POCUS. Our objective was to identify ideal pediatric appropriate POCUS equipment and standardize its use.

Methods: A Phillips Lumify probe paired with Android-Samsung tablet and a Butterfly iQ probe paired with an iPad were considered. A workflow algorithm and cleaning instructions were created. If the POCUS scan answered the clinical question, then a detailed echo was deferred. If the POCUS scan revealed findings requiring comprehensive evaluation, a follow-up echo was …

Tricuspid Atresia With Totally Anomalous Pulmonary Venous Connection: An Uncommon Finding, Anmol Goyal

Research Days

Background: Tricuspid atresia comprises 3-4% of all congenital heart disease (CHD). Anatomic findings are no true connection between the right atrium (RA) and right ventricle (RV) with concomitant findings of a ventricular septal defect (VSD), right ventricular hypoplasia, abnormalities of right ventricular outflow tract, pulmonary valve and pulmonary arteries. Common associated lesions have been described including transposition of great vessels, hypoplasia of aortic arch, double outlet ventricle or a common arterial trunk. Total anomalous pulmonary venous connection (TAPVC) is relatively rare and we present a case in addition to the six previously described cases in literature.

Case: A 33-year-old woman …

Isolated Polymorphic Ventricular Tachycardia: An Unusual Presentation Of Acute Myocarditis, Natalie S. Shwaish

Research Days

No abstract provided.

Simulating A Novel Angioplasty Technique On A 3d Printed Model​, Sarah Studyvin, Bethany Runkel

Research Days

No abstract provided.

Unusual Echocardiographic Findings Of Myocarditis Mimicking An Aortic Run-Off Lesion, Sarah Studyvin, Barbara A. Pahud, Christine Symes

Research Days

No abstract provided.

Heart Rate Recovery Following Exercise Testing In Pediatric Patients With Acyanotic Repaired Congenital Heart Disease, Natalie S. Shwaish

Research Days

No abstract provided.