Cardiology Commons^™

Medium-Term Complications Associated With Coronary Artery Aneurysms After Kawasaki Disease: A Study From The International Kawasaki Disease Registry., Brian W. Mccrindle, Cedric Manlhiot, Jane W Newburger, Ashraf S. Harahsheh, Therese M. Giglia, Frederic Dallaire, Kevin Friedman, Tisiana Low, Kyle Runeckles, Mathew Mathew, Andrew S. Mackie, Nadine F. Choueiter, Pei-Ni Jone, Shelby Kutty, Anji T. Yetman, Geetha Raghuveer, Elfriede Pahl, Kambiz Norozi, Kimberly E Mchugh, Jennifer S. Li, Sarah D De Ferranti, Nagib Dahdah, International Kawasaki Disease Registry

Manuscripts, Articles, Book Chapters and Other Papers

Background Coronary artery aneurysms (CAAs) may occur after Kawasaki disease (KD) and lead to important morbidity and mortality. As CAA in patients with KD are rare and heterogeneous lesions, prognostication and risk stratification are difficult. We sought to derive the cumulative risk and associated factors for cardiovascular complications in patients with CAAs after KD. Methods and Results A 34-institution international registry of 1651 patients with KD who had CAAs (maximum CAA Z score ≥2.5) was used. Time-to-event analyses were performed using the Kaplan-Meier method and Cox proportional hazard models for risk factor analysis. In patients with CAA Z scores ≥10, …

Efficacy Of A Novel Mitochondrial-Derived Peptide In A Porcine Model Of Myocardial Ischemia/Reperfusion Injury, Thomas E. Sharp, Zhenwei Gong, Amy Scarborough, Eric S. Goetzman, Murtuza J. Ali, Pablo Spaletra, David J. Lefer, Radhika H. Muzumdar, Traci T. Goodchild

School of Medicine Faculty Publications

With the complexities that surround myocardial ischemia/reperfusion (MI/R) injury, therapies adjunctive to reperfusion that elicit beneficial pleiotropic effects and do not overlap with standard of care are necessary. This study found that the mitochondrial-derived peptide S14G-humanin (HNG) (2 mg/kg), an analogue of humanin, reduced infarct size in a large animal model of MI/R. However, when ischemic time was increased, the infarct-sparing effects were abolished with the same dose of HNG. Thus, although the 60-min MI/R study showed that HNG cardioprotection translates beyond small animal models, further studies are needed to optimize HNG therapy for longer, more patient-relevant periods of cardiac …

Brain Abscess And The Nonfenestrated Fontan Circulation, Bethany Runkel, William B. Drake, Geetha Raghuveer

Manuscripts, Articles, Book Chapters and Other Papers

Brain abscess is a rare but life-threatening condition. Intracardiac or extracardiac right-to-left shunting in patients with unrepaired cyanotic congenital heart disease, pulmonary arteriovenous malformations, or venovenous collaterals allows microbes unfiltered access to the brain. Brain abscess must be considered when cyanotic patients present with headache.

Challenges And Special Aspects Of Pulmonary Hypertension In Middle- To Low-Income Regions: Jacc State-Of-The-Art Review, Babar Hasan, Georg Hansmann, Werner Budts, Alexandra Heath, Zahra Hoodbhoy, Zhi-Cheng Jing, Martin Koestenberger, Katharina Meinel, Ana Olga Mocumbi, Ganna D. Radchenko

Department of Paediatrics and Child Health

Challenges and special aspects related to the management and prognosis of pulmonary hypertension (PH) in middle- to low-income regions (MLIRs) range from late presentation to comorbidities, lack of resources and expertise, cost, and rare options of lung transplantation. Expert consensus recommendations addressing the specific challenges for prevention and therapy of PH in MLIRs with limited resources have been lacking. To date, 6 MLIR-PH registries containing mostly adult patients with PH exist. Importantly, the global prevalence of PH is much higher in MLIRs compared with high-income regions: group 2 PH (left heart disease), pulmonary arterial hypertension associated with unrepaired congenital heart …

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.

Atrioventricular Nodal Reentrant Tachycardia In Patients With Complex Congenital Heart Disease And Twin Atrioventricular Nodes., John Papagiannis, Christopher Mathis, Lindsey Malloy-Walton, Svjetlana Tisma-Dupanovic

Manuscripts, Articles, Book Chapters and Other Papers

No abstract provided.

Successful Completion Of Pregnancy After Mustard Procedure For Transposition Of Great Arteries: A Rare Case From The Developing World, Muhammad Kamran Younis Memon, Ayesha Malik, Saleem Akhtar

Department of Paediatrics and Child Health

Surgical repair of transposition of great arteries has undergone various evolutionary changes over the years. The initial of these treatment options was atrial septectomy followed by atrial switch and the current preferred treatment option is arterial switch operation worldwide. Due to various reasons, like lack of medical and surgical expertise atrial switch operation was commonly in practice in developing countries until few years back. Pregnancy in a patient with history of atrial switch operation poses a unique haemodynamic challenge. We report the case of a successful pregnancy in a 26 years old lady who had undergone Mustard repair surgery in …

Tetralogy Of Fallot: A Clinical Review, Jacob Wooten, Joshua Bahos Silva

Student Scholar Showcase

Tetralogy of Fallot is a congenital heart disease that is associated with structural abnormalities during fetal development 1. The incidence of Tetralogy of Fallot in the United States is approximately 3-5 cases per 10,000 live births and is considered one of the most common cyanotic congenital heart diseases 2. The exact etiology of Tetralogy of Fallot is unknown, however it is thought to be associated with untreated maternal diabetes, maternal use of retinoids, as well as chromosomal abnormalities 2. The main components of this disease state include a right ventricular outflow obstruction, interventricular communication in the form of a ventricular …

The Role Of Alternative Mrna Splicing In Heart Development, Douglas Bittel, Nataliya Kibiryeva, Naoya Kenmochi, Prakash Patil, Tamayo Uechi, Brenda Rongish, Mike Filla, Jennifer A. Marshall, Michael Artman, Rajasingh Johnson, James E. O'Brien

Manuscripts, Articles, Book Chapters and Other Papers

Research in the last 10 years has led to improved understanding of the genetic regulation of vertebrate heart development, but despite this effort, approximately 70% of all congenital heart defects (CHDs) still have an unknown etiology. Alternative splicing of mRNA has been documented to play roles in normal and abnormal development. Dysregulated splicing of mRNA has been shown to cause heart defects in mice, however a link between mRNA splicing and CHDs has not yet been shown in humans. We reported that more than 50% of genes associated with heart development were alternatively spliced in the right ventricle (RV) of …

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 …

Management Of Hypertensive Emergencies In Pediatrics, Jennifer L. Bauer, Jamie L. Amero, Amanda C. Mcdavid, Ryan A. Fischer, Alison L. Huet, Karen L. Kier

Pharmacy and Wellness Review

As hypertension becomes more prevalent in the pediatric population, clinicians are more likely to encounter hypertensive emergencies in children, which require pharmacists and physicians to be educated on the therapeutic options for these emergencies. However, the strict governmental requirements on the testing of these drugs in pediatric patients have limited the amount of available evidence on which to base clinical decisions. This review will highlight the available evidence and preferred treatment options for the management of pediatric hypertensive emergencies.

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 …

The Man With 2 Hearts: 25 Years From Heterotopic To Orthotopic Heart Transplantation, Elizabeth L Godfrey, Michael L Kueht, Abbas Rana, O H Frazier

The Texas Heart Institute Journal

Substantial technological advances in mechanical circulatory support have caused a shift in the management of end-stage heart failure. From the 1970s through the 1990s, heterotopic heart transplantation was routinely performed in patients in whom orthotopic transplantation was likely to fail. Heterotopic heart transplantation is now performed less often because modern mechanical circulatory assist devices are routinely used as bridges to orthotopic transplantation; regardless, the operation has helped numerous patients who would not otherwise have received adequate allografts. We describe the case of a man with idiopathic nonischemic cardiomyopathy who, at age 17, was given an ABO- and size-matched heterotopic allograft …

Permanent Junctional Reciprocating Tachycardia In Infants And Children, Ranjit I. Kylat Md, Ricardo A. Samson

School of Medicine Faculty Research

Permanent junctional reciprocating tachycardia (PJRT) is a rare form of supraventricular tachycardia (SVT). It generally presents in infants but can be difficult to diagnose. The characteristic EKG findings, response to Adenosine and persistence or frequent recurrences are helpful in making the diagnosis. It is usually difficult to manage with the initial and single medications used in SVT. Many patients are misdiagnosed and not treated effectively and end up having end stage cardiomyopathy and are diagnosed in patients referred for transplant. Hence all patients referred for a cardiac transplant with dilated cardiomyopathy need to be evaluated for this arrhythmia. If appropriate …

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.

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.