Cardiology Commons^™

Incidental Diagnosis Of Dual Arterial Supply Of The Right Lower Lobe, Christopher Mathis, Jay Crockett, Maria Kiaffas

Posters

Description of Clinical Presentation: A 15 year old male with a 2 week history of fever and abdominal pain had an abdominal/pelvic MRI demonstrating osteomyelitis of his right hip. Incidentally an anomalous vessel arising from the abdominal aorta and coursing to the vasculature of the lower right lung lobe was identified and he was referred to cardiology for further evaluation. He is an athlete with no history of cardiac or respiratory symptoms and his cardiac physical examination was within normal limits. Diagnostic Techniques and Their Most Important Findings: An echocardiogram was initially obtained; imaging was difficult due to suboptimal acoustic …

Prevalence Of Sensorineural Hearing Loss In Children With Palliated Or Repaired Congenital Heart Disease., Lalitha Gopineti, Mane Paulpillai, Andrea Rosenquist, Andrew H. Van Bergen

Manuscripts, Articles, Book Chapters and Other Papers

Background Children with congenital heart disease (CHD) are at increased risk of neurodevelopmental deficits, and the presence of sensorineural hearing loss (SNHL) may further lead to poor language skills acquisition and speech delays. Prevalence of SNHL in the general pediatric population is estimated to be 0.2% at birth to 0.35% during adolescence. Very few studies have attempted to estimate SNHL prevalence in children who have undergone congenital heart surgery. Methods This retrospective study aimed to estimate SNHL prevalence in children who underwent congenital heart surgery in our institution and were followed up in our high-risk pediatric cardiology clinics for four …

Fit Clinical Decision-Making: Brain Abscess In A Nonfenestrated Fontan Patient, Bethany Runkel, Natalie Shwaish, Geetha Raghuveer, William Drake

Posters

No abstract provided.

Fit Clinical Decision-Making: Isolated Polymorphic Ventricular Tachycardia In A Pediatric Patient: An Unusual Presentation Of Acute Mycocarditis, Natalie S. Shwaish, Bethany Runkel, Lindsey Malloy-Walton

Posters

No abstract provided.

Fit Clinical Decision-Making: Uhl's Anomaly: An Uncommon Cause Of Fetal Cardiomegaly, Bethany Runkel, Atif Ahmed, Tara Swanson, Maria Kiaffas

Posters

No abstract provided.

Fit Clinical Decision-Making Unusual Echocardiographic Findings Of Myocarditis Mimicking An Aortic Runoff Lesion, Sarah Studyvin, Christine Symes, Barbara A. Pahud, Nitin Madan

Posters

No abstract provided.

Anticipation, Accompaniment, And A Good Death In Perinatal Care., Bryanna S. Moore, Brian S. Carter, Bryan Beaven, Katie House, Joel House

Manuscripts, Articles, Book Chapters and Other Papers

The ethics of perinatal care, and the experiences of families who receive such care, remains a nascent area of inquiry. It can be hard to see how existing "good death" constructs apply to the experiences of fetal patients and their families. In this paper, we explore two themes raised by a case at our fetal health center: anticipation and accompaniment. In this case, a mother presented to our fetal health center; her unborn son, our fetal patient, was diagnosed with life-threatening hypoplastic left heart syndrome and endocardial fibroelastosis. The parents were told that their son's life expectancy, upon birth, was …

The Early "Unnatural" History Following Surgical Repair Of Ventricular Septal Defects., Sathish M. Chikkabyrappa, Justin T. Tretter, Arpan R. Doshi, Sujatha Buddhe, Puneet Bhatla, Achi Ludomirsky

Manuscripts, Articles, Book Chapters and Other Papers

Introduction: Surgical outcomes for simple ventricular septal defects (VSD) have been excellent in the past three decades. For this project, the timing of resolution of left-sided dilation and mitral regurgitation (MR) following VSD repair was assessed.

Methods: Echocardiographic data surrounding surgery of 42 consecutive children who underwent surgical patch repair of a VSD were reviewed. The echocardiograms were reviewed up to a mean of 12 months post-operatively (range 9 - 14 months). Quantitative data indexed to body surface area including left atrial (LA) volume, mitral valve annulus diameter, and left ventricular end-diastolic dimension (LVEDD) was analyzed.

Results: The majority of …

Failure To Rescue As An Outcome Metric For Pediatric And Congenital Cardiac Catheterization Laboratory Programs: Analysis Of Data From The Impact Registry., Michael L. O'Byrne, Kevin F. Kennedy, Natalie Jayaram, Lisa J. Bergersen, Matthew J. Gillespie, Yoav Dori, Jeffrey H. Silber, Steven M. Kawut, Jonathan J. Rome, Andrew C. Glatz

Manuscripts, Articles, Book Chapters and Other Papers

Background Risk-adjusted adverse event (AE) rates have been used to measure the quality of pediatric and congenital cardiac catheterization laboratories. In other settings, failure to rescue (FTR) has demonstrated utility as a quality metric. Methods and Results A multicenter retrospective cohort study was performed using data from the IMPACT (Improving Adult and Congenital Treatment) Registry between January 2010 and December 2016. A modified FTR metric was developed for pediatric and congenital cardiac catheterization laboratories and then compared with pooled AEs. The associations between patient- and hospital-level factors and outcomes were evaluated using hierarchical logistic regression models. Hospital risk standardized ratios …

Comparison Of Echocardiographic Measurements To Invasive Measurements Of Diastolic Function In Infants With Single Ventricle Physiology: A Report From The Pediatric Heart Network Infant Single Ventricle Trial., Suma P. Goudar, Victor Zak, Andrew M. Atz, Karen Altmann, Steven D. Colan, Christine B. Falkensammer, Mark K. Friedberg, Michele Frommelt, Kevin D. Hill, Daphne T. Hsu, Jami C. Levine, Renee Margossian, Christopher R. Mart, Joshua Sticka, Peter Shrader, Girish S. Shirali, Pediatric Heart Network Investigators

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: While echocardiographic parameters are used to quantify ventricular function in infants with single ventricle physiology, there are few data comparing these to invasive measurements. This study correlates echocardiographic measures of diastolic function with ventricular end-diastolic pressure in infants with single ventricle physiology prior to superior cavopulmonary anastomosis.

METHODS: Data from 173 patients enrolled in the Pediatric Heart Network Infant Single Ventricle enalapril trial were analysed. Those with mixed ventricular types (n = 17) and one outlier (end-diastolic pressure = 32 mmHg) were excluded from the analysis, leaving a total sample size of 155 patients. Echocardiographic measurements were correlated to …

A Population Pharmacokinetic Model For Simvastatin And Its Metabolites In Children And Adolescents., Kayode Ogungbenro, Jonathan B. Wagner, Susan M. Abdel-Rahman, J Steven Leeder, Aleksandra Galetin

Manuscripts, Articles, Book Chapters and Other Papers

PURPOSE: Poor adherence to dietary/behaviour modifications as interventions for hypercholesterolemia in paediatric patients often necessitates the initiation of statin therapy. The aim of this study was to develop a joint population pharmacokinetic model for simvastatin and four metabolites in children and adolescents to investigate sources of variability in simvastatin acid exposure in this patient population, in addition to SLCO1B1 genotype status.

METHODS: Plasma concentrations of simvastatin and its four metabolites, demographic and polymorphism data for OATP1B1 and CYP3A5 were analysed utilising a population pharmacokinetic modelling approach from an existing single oral dose (10 mg < 17 years and 20 mg ≥ 18 years) pharmacokinetic dataset of 32 children and adolescents.

RESULTS: The population PK model included …

Electromechanical Dyssynchrony And Clinically Silent Ventricular Dysfunction In Young Subjects With Ventricular Pacing For Congenital And Early Acquired Av Block, Daniel Forsha, David G. Gamboa, Niels Risum, Andrea Kropf, Christoph P. Hornik, Piers Barker, Joseph Kisslo, Ronald J. Kanter

Manuscripts, Articles, Book Chapters and Other Papers

Purpose: Single site ventricular pacing can result in electromechanical dyssynchrony and LV dysfunction. A classic pattern by strain echocardiography pattern analysis has been defined that specifically identifies electromechanical dyssynchrony that leads to LV dysfunction in other populations. Our purpose is to evaluate for the presence of the classic pattern and an association with LV dysfunction in asymptomatic young subjects with chronic pacing.

Methods: Inclusion criteria of non-surgical 2nd or 3rd degree AV block, normal cardiac anatomy, chronic ventricular pacing (> 95% of beats) and a quality 4-chamber apical image were met by 24 retrospective and 10 prospectively enrolled subjects. Global …

Effect Of Intravenous Phenobarbital On Left Ventricular Myocardial Contractility Determined By Echocardiography In Children., Arpan R. Doshi, Monesha Gupta Malhotra, Duraisamy Balaguru, Devendra Amre, Christian Erikson

Manuscripts, Articles, Book Chapters and Other Papers

Introduction: Animal studies and rare human studies have suggested a negative effect of barbiturates on cardiac function. Although intravenous (IV) phenobarbital is used routinely in children in the clinical setting, studies in children are lacking. We performed a study to evaluate effect of IV phenobarbital loading on myocardial systolic function of children.

Methods: In a prospective pilot study in children without congenital heart defects, the effect of IV phenobarbital was evaluated on the left ventricular systolic function measured by ejection fraction (EF) by Simpson's method via an echocardiogram. Any child less than 18 years of age who received IV loading …

Av Nodal Reentrant Tachycardia In Patients With Twin Av Nodes, Christopher Mathis, Svjetlana Tisma-Dupanovic, Lindsey Malloy-Walton, John Papagiannis

Posters

No abstract provided.

Practice Variations In Sedated Echocardiography And Impact On Repeat Echocardiography In Non-Refractory Kawasaki Disease, Nicholas Clark, Troy Richardson, Jennifer E. Schuster, Anitha Parthiban, Henry T. Puls

Posters

This study discovered significant variation among children's hospitals in the use of sedated echocardiiography for children <30 months of age with non-refractory Kawasaki disease prior to the American Heart Association 2017 Kawasake disease guideline update. Consideration should be given to performing sedated echocardiography as it may reduce the need for repeat echos without impacting length of stay or total costs.

Ucp1 Expression-Associated Gene Signatures Of Human Epicardial Adipose Tissue., Kanta Chechi, Jinchu Vijay, Pierre Voisine, Patrick Mathieu, Yohan Bossé, Andre Tchernof, Elin Grundberg, Denis Richard

Manuscripts, Articles, Book Chapters and Other Papers

Multiple reports of uncoupling protein 1 (UCP1) expression have established its presence in human epicardial adipose tissue (eAT). Its functional relevance to eAT, however, remains largely unknown. In a recent study, we reported that adrenergic stimulation of eAT was associated with downregulation of secreted proteins involved in oxidative stress-related and immune-related pathways. Here, we explored the UCP1-associated features of human eAT using next-generation deep sequencing. Paired biopsies of eAT, mediastinal adipose tissue (mAT), and subcutaneous adipose tissue (sAT) obtained from cardiac surgery patients, with specific criteria of high and low expression of UCP1 in eAT, were subjected to RNA sequencing. …

Identification Of Inadvertent Azygous Vein Cannulation Using Transthoracic Echocardiography During Venoarterial Extracorporeal Membrane Oxygenation Initiation., Bethany Runkel, Jason D. Fraser, John Daniel, Karina M. Carlson

Manuscripts, Articles, Book Chapters and Other Papers

It is well known that optimal cannula positioning during initiation of venoarterial (VA) extracorporeal membrane oxygenation (ECMO) is directly related to ECMO circuit function. Malpositioned cannulae can have deleterious effects on clinical outcomes, and inadvertent cannulation of the azygous vein during initiation of ECMO is a rare but potentially devastating complication that has been reported only a few times in the literature. Here we report a case of azygous vein cannulation in a neonate that was not identified on chest radiography but was recognized and corrected expeditiously with the use of transthoracic echocardiography.

Choosing Catheter Sheath Introducers In Infants-Information You Are Not Provided But Should Have!, Christopher Mathis, Ryan Romans, Abhay Divekar

Posters

Background: The advertised catheter sheath introducer (CSI) size, the internal diameter (ID), defines the maximum size of medical devices that can pass through it. However, the outer diameter (OD) determines the risk of vascular injury, varies considerably between manufacturers, and is not advertised. The highest risk of vascular injury is in infants less than one year of age and 3 - 6Fr CSIs are typically used.

Methods: The OD of 3, 3.3, 4, 5, and 6F CSIs (5 of each size) from different manufacturers were measured using a Mitutoyo Digital Micrometer. CSIs were measured with the dilator in place. Three …

Coarctation Of Aorta In Children., Arpan R. Doshi, Sathish Chikkabyrappa

Manuscripts, Articles, Book Chapters and Other Papers

Coarctation of aorta (CoA) is a discrete narrowing in aorta causing obstruction to the flow of blood. It accounts for 6-8% of all congenital heart diseases. With advances in fetal echocardiography rate of prenatal diagnosis of coarctation of aorta has improved but it still remains a challenging diagnosis to make prenatally. Transthoracic echocardiography is mainstay of making initial diagnosis and routine follow-up. Cardiac magnetic resonance imaging (MRI) and computed tomography (CT) are great advanced imaging tools for two-dimensional and three-dimensional imaging of aortic arch in complex cases. Based on type of coarctation, size of patient, severity of lesion, and associated …

Innocent Heart Murmur., Arpan R. Doshi

Manuscripts, Articles, Book Chapters and Other Papers

Heart murmur is the most common reason for a referral to a pediatric cardiologist. Virtually all children have a heart murmur during their childhood. Less than 1% of murmurs are pathological in children. Innocent/functional heart murmur is the most common type of heart murmur. There are multiple theories proposed to identify etiology of innocent heart murmur with varying consensus, but everybody agrees that innocent heart murmur does not carry any morbidity or mortality risk. Even today, heart murmur is associated with high physician uncertainty and parental anxiety. Extensive cardiac evaluation for such a benign finding is also associated with high …

The Role Of Scarnas In Adjusting Alternative Mrna Splicing In Heart Development., Chloe Nagasawa, Allison Ogren, Nataliya Kibiryeva, Jennifer A. Marshall, James O'Brien, Naoya Kenmochi, Douglas C. Bittel

Manuscripts, Articles, Book Chapters and Other Papers

Congenital heart disease (CHD) is a leading cause of death in childrenyears, most CHDs (~70%) still have an unknown etiology. Conotruncal based defects, such as Tetralogy of Fallot (TOF), a common complex of devastating heart defects, typically requires surgical intervention in the first year of life. We reported that the noncoding transcriptome in myocardial tissue from children with TOF is characterized by significant variation in levels of expression of noncoding RNAs, and more specifically, a significant reduction in 12 small cajal body-associated RNAs (scaRNAs) in the right ventricle. scaRNAs are essential for the biochemical modification and maturation of small nuclear …

Longitudinal Measures Of Deformation Are Associated With A Composite Measure Of Contractility Derived From Pressure-Volume Loop Analysis In Children., Shahryar M. Chowdhury, Ryan J. Butts, Carolyn L. Taylor, Varsha M. Bandisode, Karen S. Chessa, Anthony M. Hlavacek, Arni Nutting, Girish S. Shirali, G Hamilton Baker

Manuscripts, Articles, Book Chapters and Other Papers

Aims: The relationship between echocardiographic measures of left ventricular (LV) systolic function and reference-standard measures have not been assessed in children. The objective of this study was to assess the validity of echocardiographic indices of LV systolic function via direct comparison to a novel composite measure of contractility derived from pressure-volume loop (PVL) analysis.

Methods and results: Children with normal loading conditions undergoing routine left heart catheterization were prospectively enrolled. PVLs were obtained via conductance catheters. A composite invasive composite contractility index (ICCI) was developed using data reduction strategies to combine four measures of contractility derived from PVL analysis. Echocardiograms …

Echocardiographic Detection Of Increased Ventricular Diastolic Stiffness In Pediatric Heart Transplant Recipients: A Pilot Study., Shahryar M. Chowdhury, Ryan J. Butts, Anthony M. Hlavacek, Carolyn L. Taylor, Karen S. Chessa, Varsha M. Bandisode, Girish S. Shirali, Arni Nutting, G Hamilton Baker

Manuscripts, Articles, Book Chapters and Other Papers

Background: Pediatric heart transplant recipients are at risk for increased left ventricular (LV) diastolic stiffness. However, the noninvasive evaluation of LV stiffness has remained elusive in this population. The objective of this study was to compare novel echocardiographic measures of LV diastolic stiffness versus gold-standard measures derived from pressure-volume loop (PVL) analysis in pediatric heart transplant recipients.

Methods: Patients undergoing left heart catheterization were prospectively enrolled. PVLs were obtained via conductance. The end-diastolic pressure-volume relationship was obtained via balloon occlusion. The stiffness constant, β, was calculated. Echocardiographic measures of diastolic function were derived from spectral and tissue Doppler and two-dimensional …

The World Database For Pediatric And Congenital Heart Surgery: Use Of An International Congenital Database In South Korea., James D. St Louis, Cheul Lee, Hiromi Kurosawa, Richard A. Jonas, Sakamoto Kisaburo, Christo I. Tchervenkov, Jeffery P. Jacobs, James K. Kirklin

Manuscripts, Articles, Book Chapters and Other Papers

No abstract provided.

Implementing Lean Daily Management System To Improve Cvor First Case On-Time Starts, Haley Borchers, Kelly Fehlhafer, Barbara Mueller, Jessica Nichols, Sarah Talken, Mary Hunter, Kenneth Sam

Posters

No abstract provided.

Decreasing Cardiac Arrests In The Pediatric Intensive Care Unit, Jeremy Affolter, Kathlyn Baharaeen, Mari Hanson, Lisa Laddish, Amy Bohm, Lindsey Bradbury, Megan Mcghee, Anne Leroy, Hannah Cunningham, Michelle Waddell, Tiffany Mullen, Kimberly Lucas, William Douglas, Angel Pope, Marita Thompson, Paul N. Bauer, Erica Molitor-Kirsch, Tara Benton, Laura Miller-Smith

Posters

No abstract provided.

Knowledge Base Of Adolescents With Congenital Heart Disease, Pamela Finn, Mark Gelatt, Jennifer A. Marshall, Jennifer Panuco, Jenea Schmidt

Posters

Introduction:

Most congenital heart disease [CHD] is diagnosed and treated in early childhood in designated children’s hospitals with parents responsible for decision-making and receipt of information. The adolescent assumes this role in preparation for transition to an adult congenital heart program. We studied the knowledge base of our adolescent CHD patients and their parents.

Methods:

Established patients with CHD, >11 y.o. and their parents, were independently surveyed in the outpatient clinic prior to being seen over a one-year period. Participation was voluntary. Cardiomyopathy, transplant and electrophysiology patients were excluded. Scores were assessed as full, partial or incomplete.

Results:

Most (98% …

Knowledge Base Of Adolescents With Congenital Heart Disease, Mark Gelatt, Julie Martin, Jennifer A. Marshall, Jennifer Panuco, Jenea Schmidt, Pamela Finn

Posters

Introduction:

Most congenital heart disease [CHD] is diagnosed and treated in early childhood with parents responsible for decision-making. The adolescent assumes this role in preparation for transition to an adult congenital heart program. We studied the knowledge base of our adolescent CHD patients and their parents.

Methods:

Established CHD patients, >11 y.o. and their parents, were independently surveyed in the outpatient clinic. Participation was voluntary. Cardiomyopathy, electrophysiology and transplant patients were excluded. Scores were assessed as full, partial or incomplete.

Results:

Most (98% parents; 83% adolescents) reported that their cardiologist had provided education. Adolescents provided a full (49%) and partial …

Congenital Left Ventricle-To-Coronary Sinus Fistula: A Rare Isolated Anomaly Of The Coronary Sinus., Doaa M. Aly, Nikki M. Singh, Sanket Shah

Manuscripts, Articles, Book Chapters and Other Papers

Congenital anomalies of the coronary sinus (CS) are very uncommon. They can occur either in isolation or in association with other congenital heart defects. 1 Among those anomalies, congenital fistulae connecting the left ventricle and the CS represent a very rare entity. Most reported cases of CS fistula generally originate in the right coronary artery or left anterior descending coronary artery system. 2 We describe an extremely rare case of an isolated high-pressure congenital left ventricle–to–CS fistula as an anomaly of the CS in an otherwise asymptomatic child. The initial clinical presentation and follow-up, imaging studies, and plan for management …

Genomic Contraindications For Heart Transplantation., Danton S. Char, Gabriel Lázaro-Muñoz, Aliessa Barnes, David Magnus, Michael J. Deem, John Lantos

Manuscripts, Articles, Book Chapters and Other Papers

Genome sequencing raises new ethical challenges. Decoding the genome produces new forms of diagnostic and prognostic information; however, the information is often difficult to interpret. The connection between most genetic variants and their phenotypic manifestations is not understood. This scenario is particularly true for disorders that are not associated with an autosomal genetic variant. The analytic uncertainty is compounded by moral uncertainty about how, exactly, the results of genomic testing should influence clinical decisions. In this Ethics Rounds, we present a case in which genomic findings seemed to play a role in deciding whether a patient was to be listed …