Pediatrics Commons^™

A Rare Case Of Vascular Ring And Coarctation Of The Aorta In Association With Charge Syndrome., Jonathan B. Wagner, Joshua Q. Knowlton, Peter Pastuszko, Sanket Shah

Manuscripts, Articles, Book Chapters and Other Papers

A male neonate presented with CHARGE syndrome, a multiorgan genetic disorder involving the Coloboma of the eyes, congenital Heart defects, nasal choanal Atresia, growth and development Retardation, Genitourinary disorders, and Ear anomalies and deafness. Moreover, he had a rare case of vascular ring-consisting of a right aortic arch with retroesophageal brachiocephalic artery-combined with coarctation of the mid-aortic arch. He underwent both vascular ring and aortic arch repair at our institution. To our knowledge, this is the 4th documented case of this exceedingly rare type of aortic arch anomaly combined with aortic arch obstruction. Moreover, it is the first confirmed case …

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 …

Usage Of 3d Models Of Tetralogy Of Fallot For Medical Education: Impact On Learning Congenital Heart Disease., Yue-Hin Loke, Ashraf S Harahsheh, Axel Krieger, Laura J Olivieri

Pediatrics Faculty Publications

BACKGROUND: Congenital heart disease (CHD) is the most common human birth defect, and clinicians need to understand the anatomy to effectively care for patients with CHD. However, standard two-dimensional (2D) display methods do not adequately carry the critical spatial information to reflect CHD anatomy. Three-dimensional (3D) models may be useful in improving the understanding of CHD, without requiring a mastery of cardiac imaging. The study aimed to evaluate the impact of 3D models on how pediatric residents understand and learn about tetralogy of Fallot following a teaching session.

METHODS: Pediatric residents rotating through an inpatient Cardiology rotation were recruited. The …

A Structured Blood Conservation Program In Pediatric Cardiac Surgery, A Budak, Kevin Mccusker, S Gunaydin

NYMC Faculty Publications

OBJECTIVE: The limitation of alternative transfusion practices in infants increases the benefits of blood conservation. We analyzed the efficacy of a structured program to reduce transfusions and transfusion-associated complications in cardiac surgery

PATIENTS AND METHODS: Our pediatric surgery database was reviewed retrospectively, comparing outcomes from two different time periods, after the implementation of an effective blood conservation program beginning in March 2014. A total of 214 infants (8.1±3.4 months) who underwent biventricular repair utilizing CPB (Group 1 – Blood conservation) were studied in a 12-month period (March 2014-February 2015) after the implementation of the new program, and compared with 250 …

External Cardioversion Of Supraventricular Tachycardia In Omphalo-Thoracopagus Conjoined Twins., M Rizwan Afzal, Lindsey Malloy-Walton, Svjetlana Tisma-Dupanovic, Andrea L. Miles, Sanket Shah, John Papagiannis

Manuscripts, Articles, Book Chapters and Other Papers

No abstract provided.

Junctional Ectopic Tachycardia Secondary To Myocarditis Associated With Sudden Cardiac Arrest, Michael E. A. Cunningham, Robin W. Doroshow, Laura Oliveri, Jeffrey P. Moak

Pediatrics Faculty Publications

No abstract provided.

Titanium Plug Closure After Heartware Ventricular Assist Device Explantation In A 15-Year-Old Girl: First Us Experience, Diego A Lara, Aamir Jeewa, Barbara A Elias, Elizabeth O Mccullum, Susan W Denfield, William J Dreyer, Iki Adachi

The Texas Heart Institute Journal

We describe the case of a teenage girl with anthracycline-induced cardiomyopathy who received a HeartWare ventricular assist device and underwent successful device explantation after cardiac recovery. During device support, the patient's cardiac function returned to normal. Twelve months after implantation, we explanted the device via repeat median sternotomy. To close the hole in the left ventricular apex and preserve the sewing ring in case future device support is needed, we used a German-manufactured titanium plug, developed specifically for this purpose. To our knowledge, this is the first use of this plug in the United States. The patient recovered uneventfully and …

Pediatric Cardiovascular Care In Uganda: Current Status, Challenges, And Opportunities For The Future., Twalib Olega Aliku, Sulaiman Lubega, Judith Namuyonga, Tom Mwambu, Michael Oketcho, John O Omagino, Craig Sable, Peter Lwabi

Pediatrics Faculty Publications

In many developing countries, concerted action against common childhood infectious diseases has resulted in remarkable reduction in infant and under-five mortality. As a result, pediatric cardiovascular diseases are emerging as a major contributor to childhood morbidity and mortality. Pediatric cardiac surgery and cardiac catheterization interventions are available in only a few of Sub-Saharan African countries. In Uganda, open heart surgeries (OHSs) and interventional procedures for pediatric cardiovascular disease are only possible at the Uganda Heart Institute (UHI), having been started with the help of expatriate teams from the years 2007 and 2012, respectively. Thereafter, independent OHS and cardiac catheterization have …

Pre-Implementation Of A Fontan Post-Operative Clinical Pathway: Summary Of 2016 Pdsa Cycles, Sarah M. Lagergren, Bryan Beaven, Suma Goudar, Megan Jensen

Posters

Background/Introduction: Post-operative hospitalization for the Fontan procedure tends to have an extended hospital length of stay (LOS). From 2013-2014, the average LOS at Children’s Mercy for the Fontan procedure is 14 days, whereas the U.S. national reported average is 11 days. Post-operative management of this patient population is often caregiver dependent. It has been theorized that developing a more standardized post-operative management regimen tailored specifically toward the unique physiology of Fontan patients may be able to improve outcomes and decrease LOS.

Methods: A review of literature was performed and revealed three pediatric institutions have published their post-operative Fontan care guidelines. …

Speckle-Tracking Echocardiographic Measures Of Right Ventricular Diastolic Function Correlate With Reference Standard Measures Before And After Preload Alteration In Children., Shahryar M. Chowdhury, Suma Goudar, G Hamilton Baker, Carolyn L. Taylor, Girish S. Shirali, Mark K. Friedberg, Andreea Dragulescu, Karen S. Chessa, Luc Mertens

Manuscripts, Articles, Book Chapters and Other Papers

The accuracy of echocardiographic measures of right ventricular (RV) diastolic function has been sparsely studied. Our objective was to evaluate the correlation between echocardiographic and reference standard measures of RV diastolic function derived from micromanometer pressure analysis before and after preload alteration in children. Echocardiograms and micromanometer pressure analyses were prospectively performed before and after fluid bolus in children undergoing right heart catheterization. The isovolumic relaxation time constant (τ) and end-diastolic pressure (EDP) were measured. Conventional and speckle-tracking echocardiographic (STE) parameters of RV systolic and diastolic function were assessed. Normal saline bolus was given to increase RV EDP by 20 …

Relationship Between Hospital Procedure Volume And Complications Following Congenital Cardiac Catheterization: A Report From The Improving Pediatric And Adult Congenital Treatment (Impact) Registry., Natalie Jayaram, John A. Spertus, Michael L. O'Byrne, Paul S. Chan, Kevin F. Kennedy, Lisa Bergersen, Andrew C. Glatz

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: The association between institutional volume and outcomes has been demonstrated for cardiac catheterization among adults, but less is known about this relationship for patients with congenital heart disease (CHD) undergoing cardiac catheterization.

METHODS: Within the IMPACT registry, we identified all catheterizations between January 2011 and March 2015. Hierarchical logistic regression, adjusted for patient and procedural characteristics, was used to determine the association between annual catheterization lab volume and occurrence of a major adverse event (MAE).

RESULTS: Of 56,453 catheterizations at 77 hospitals, an MAE occurred in 1014 (1.8%) of cases. In unadjusted analysis, a MAE occurred in 2.8% (123/4460) …

Mechanisms Of Vasovagal Syncope In The Young: Reduced Systemic Vascular Resistance Versus Reduced Cardiac Output, Julian Stewart, Marvin Medow, R Sutton, Paul Visintainer, D Jardine, W Wieling

NYMC Faculty Publications

BACKGROUND: Syncope is a sudden transient loss of consciousness and postural tone caused by cerebral hypoperfusion. The most common form is vasovagal syncope (VVS). Presyncopal progressive early hypotension in older VVS patients is caused by reduced cardiac output (CO); younger patients have reduced systemic vascular resistance (SVR). Using a priori criteria for reduced CO ( downward arrowCO) and SVR ( downward arrowSVR), we studied 48 recurrent young fainters comparing subgroups of VVS with VVS- downward arrowCO, VVS- downward arrowSVR, and both VVS- downward arrowCO& downward arrowSVR. METHODS AND RESULTS: Subjects were studied supine and during 70-degrere upright tilt with a …

A Quality Improvement Project For The Evaluation And Management Of Possible Necrotizing Enterocolitis In Single Ventricle Patients, Laura Miller-Smith, Jennifer Flint, Lisa Laddish

Posters

Background:

Infants with single ventricle (SV) physiology are at high risk for interstage complications, including necrotizing enterocolitis (NEC). The underlying pathophysiology and age at presentation differs from NEC literature described in premature infants, making NEC in SV patients challenging to diagnose and treat. We perceive significant variability in the approach to possible NEC in SV patients at our institution.

Aims:

To identify variability & standardize the approach to management of NEC

To create a management algorithm for diagnosing and treating NEC

Methods:

After review of current practice and review of medical literature, an algorithm to guide medical decision making specific …

Obstacles To Introducing A Post-Operative Feeding Protocol In The Single Ventricle Population, Lisa Laddish, Laura Miller-Smith, Jennifer Flint

Posters

Objective:

To decrease the morbidity associated with feeding complications by implementing a post-operative feeding protocol for infants with single ventricle physiology

Interpreting Measurements Of Cardiac Function Using Vendor-Independent Speckle Tracking Echocardiography In Children: A Prospective, Blinded Comparison With Catheter-Derived Measurements., Suma Goudar, G Hamilton Baker, Shahryar M. Chowdhury, Kimberly J. Reid, Girish S. Shirali, Mark A. Scheurer

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Adult studies demonstrate that echocardiographic measurements of cardiac function using speckle tracking correlate with invasive measurements, but such data in the pediatric population are sparse. Our aim was to compare speckle-derived measures of cardiac function to measurements routinely obtained by cardiac catheterization in children.

METHODS: Echocardiograms were performed on the day of cardiac catheterization. Using Tomtec 2D Cardiac Performance Analysis, longitudinal strain (LS), longitudinal strain rate (LSR), early diastolic LSR, and ejection fraction (EF) for the right and left ventricle (RV and LV) were calculated via speckle tracking. Global LS and LSR were calculated for the LV. These results …

Parental Refusal Of Surgery In An Infant With Tricuspid Atresia., Alexander A. Kon, Angira Patel, Steven Leuthner, John Lantos

Manuscripts, Articles, Book Chapters and Other Papers

We present a case of a fetal diagnosis of tricuspid atresia (TA). The pregnant woman and her husband requested that the baby be treated with only palliative care. The cardiologist did not think it would be appropriate to withhold life-prolonging surgery once the infant was born. The neonatologist argued that outcomes for TA are similar to those for hypoplastic left heart syndrome, and the standard practice at the institution was to allow parents to choose surgery or end-of-life care for those infants. The team requested an ethics consultation to assist in determining whether forgoing life-prolonging interventions in this case would …

Assessment Of Diastolic Function In Single-Ventricle Patients After The Fontan Procedure., Renee Margossian, Lynn A. Sleeper, Gail D. Pearson, Piers C. Barker, Luc Mertens, Michael D. Quartermain, Jason T. Su, Girish S. Shirali, Shan Chen, Steven D. Colan, Pediatric Heart Network Investigators

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Patients with functional single ventricles after the Fontan procedure have abnormal cardiac mechanics. The aims of this study were to determine factors that influence diastolic function and to describe associations of diastolic function with current clinical status.

METHODS: Echocardiograms were obtained as part of the Pediatric Heart Network Fontan Cross-Sectional Study. Diastolic function grade (DFG) was assessed as normal (grade 0), impaired relaxation (grade 1), pseudonymization (grade 2), or restrictive (grade 3). Studies were also classified dichotomously (restrictive pattern present or absent). Relationships between DFG and pre-Fontan variables (e.g., ventricular morphology, age at Fontan, history of volume-unloading surgery) and …

Rationale And Design Of The Children's Oncology Group (Cog) Study Alte1621: A Randomized, Placebo-Controlled Trial To Determine If Low-Dose Carvedilol Can Prevent Anthracycline-Related Left Ventricular Remodeling In Childhood Cancer Survivors At High Risk For Developing Heart Failure., Saro H. Armenian, Melissa M. Hudson, Ming Hui Chen, Steven D. Colan, Lanie Lindenfeld, George Mills, Aida Siyahian, Sarah Gelehrter, Ha Dang, Wendy Hein, Daniel M M. Green, Leslie L. Robison, F Lennie Wong, Pamela S. Douglas, Smita Bhatia

Manuscripts, Articles, Book Chapters and Other Papers

Background: Anthracyclines are widely used in the treatment of childhood cancer. One of the well-recognized side-effects of anthracycline therapy is dose-dependent cardiomyopathy that may progress to heart failure (HF) years after completion of cancer-directed therapy. This study will evaluate the efficacy of low-dose beta-blocker (carvedilol) for HF risk reduction in childhood cancer survivors at highest risk for HF. The proposed intervention has the potential to significantly reduce chronic cardiac injury via interruption of neurohormonal systems responsible for left ventricular (LV) remodeling, resulting in improved cardiac function and decreased risk of HF. The intervention is informed by previous studies demonstrating efficacy …

An Interactive Videogame Designed To Improve Respiratory Navigator Efficiency In Children Undergoing Cardiovascular Magnetic Resonance, Sean M. Hamlet, Christopher M. Haggerty, Jonathan D. Suever, Gregory J. Wehner, Jonathan D. Grabau, Kristin N. Andres, Moriel H. Vandsburger, David K. Powell, Vincent L. Sorrell, Brandon K. Fornwalt

Saha Cardiovascular Research Center Faculty Publications

Background: Advanced cardiovascular magnetic resonance (CMR) acquisitions often require long scan durations that necessitate respiratory navigator gating. The tradeoff of navigator gating is reduced scan efficiency, particularly when the patient’s breathing patterns are inconsistent, as is commonly seen in children. We hypothesized that engaging pediatric participants with a navigator-controlled videogame to help control breathing patterns would improve navigator efficiency and maintain image quality.

Methods: We developed custom software that processed the Siemens respiratory navigator image in real-time during CMR and represented diaphragm position using a cartoon avatar, which was projected to the participant in the scanner as visual feedback. The …

Pediatric Statin Administration: Navigating A Frontier With Limited Data., Jonathan B. Wagner, Susan M. Abdel-Rahman

Manuscripts, Articles, Book Chapters and Other Papers

Increasingly, children and adolescents with dyslipidemia qualify for pharmacologic intervention. As they are for adults, 3-hydroxy-3-methyl-glutaryl-coenzyme A reductase inhibitors (statins) are the mainstay of pediatric dyslipidemia treatment when lifestyle modifications have failed. Despite the overall success of these drugs, the magnitude of variability in dose-exposure-response profiles contributes to adverse events and treatment failure. In children, the cause of treatment failures remains unclear. This review describes the updated guidelines for screening and management of pediatric dyslipidemia and statin disposition pathway to assist the provider in recognizing scenarios where alterations in dosage may be warranted to meet patients' specific needs.

Cardiac Arrest In An Adolescent With Uhl’S Anomaly: Two Unusual Cardiac Arrhythmia Mechanisms, Jeffrey P. Moak, Gail D. Pearson, Bradley Clark, Charles I. Berul, Russell R. Cross, Dilip S. Nath

Pediatrics Faculty Publications

No abstract provided.

Intracardiac Tumor Causing Left-Ventricular Outflow-Tract Obstruction In A Newborn, Christopher P. Jordan, John P. Costello, Kendal Endicott, Christine Reyes, Thomas Hougen, Susan Cummings, Dilip S. Nath

Pediatrics Faculty Publications

The following report describes the case of newborn girl with an asymptomatic systolic murmur, which on imaging revealed a nearly obstructive mass in the left-ventricular outflow tract. The mass was resected and found to be consistent with a rhabdomyoma. Here, we describe the pathologic and clinical characteristics of this tumor.

Validation Of Noninvasive Measures Of Left Ventricular Mechanics In Children: A Simultaneous Echocardiographic And Conductance Catheterization Study., Shahryar M. Chowdhury, Ryan J. Butts, Carolyn L. Taylor, Varsha M. Bandisode, Karen S. Chessa, Anthony M. Hlavacek, Girish S. Shirali, G Hamilton Baker

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: The accuracy of echocardiography in evaluating left ventricular contractility has not been validated in children. The objective of this study was to compare echocardiographic measures of contractility with those derived from pressure-volume loop (PVL) analysis in children.

METHODS: Patients with relatively normal loading conditions undergoing routine left heart catheterization were prospectively enrolled. PVLs were obtained via conductance catheters. The gold-standard measure of contractility, end-systolic elastance (Ees), was obtained via balloon occlusion of one or both vena cavae. Echocardiograms were performed immediately after PVL analysis under the same anesthetic conditions. Single-beat estimations of echocardiographic Ees were calculated using four different …

Asymptomatic Left Ventricular Myxoma In A 12-Year-Old Male., Meghan Chlebowski, James O'Brien, Casey Hertzenberg, Jonathan B. Wagner

Manuscripts, Articles, Book Chapters and Other Papers

Cardiac myxoma is the most common cardiac tumor in patients of all ages; the majority are encountered as single left atrial tumors. Left ventricular myxomas are exceedingly rare, having been recorded in a small number of case reports involving children worldwide. We report a case of a left ventricular myxoma with left ventricular outflow tract obstruction in a previously healthy, asymptomatic adolescent black male. Transthoracic echocardiograms revealed a single, large (2.5 × 5-cm), lobulated, mobile mass within the left ventricular cavity that oscillated into the outflow tract, thereby causing moderate obstruction during systole. Advanced images delineated the location and tissue …

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 …

Cardiac Remodeling And Dysfunction In Childhood Obesity: A Cardiovascular Magnetic Resonance Study, Linyuan Jing, Cassi M. Binkley, Jonathan D. Suever, Nivedita Umasankar, Christopher M. Haggerty, Jennifer Rich, Christopher D. Nevius, Gregory J. Wehner, Sean M. Hamlet, David K. Powell, Aurelia Radulescu, H. Lester Kirchner, Frederick H. Epstein, Brandon K. Fornwalt

Pediatrics Faculty Publications

Background: Obesity affects nearly one in five children and is associated with increased risk of premature death. Obesity-related heart disease contributes to premature death. We aimed to use cardiovascular magnetic resonance (CMR) to comprehensively characterize the changes in cardiac geometry and function in obese children.

Methods and results: Forty-one obese/overweight (age 12 ± 3 years, 56 % female) and 29 healthy weight children (age 14 ± 3 years, 41 % female) underwent CMR, including both standard cine imaging and displacement encoded imaging, for a complete assessment of left ventricular (LV) structure and function. After adjusting for age, LV mass index …

Childhood Psychosocial Determinants Of Cardiovascular Health, Samuel S. Gidding, Erica D. Sood

Department of Medicine Faculty Papers

Understanding risk and protective factors that impact cardiovascular health is of utmost importance. There is ample evidence that cardiovascular health begins in childhood, tracks over time, and is subject to adverse social influences. This paper reviews key studies examining the relations of psychosocial factors in childhood to cardiovascular health in adulthood. The existing literature provides evidence for both individual and cumulative effects of childhood psychosocial factors on adult cardiovascular health across the population, although the specific mechanisms underlying these relationships are not yet fully understood. This paper also includes a discussion of evidence-based strategies for prevention and treatment of childhood …

Harnessing Teams And Technology To Improve Outcomes In Infants With Single Ventricle., Girish S. Shirali, Lori A. Erickson, Johnathan Apperson, Kathy Goggin, David D. Williams, Kimberly J. Reid, Andrea Bradley-Ewing, Dawn Tucker, Michael Bingler, John Spertus, Leslie Rabbitt, Richard Stroup

Manuscripts, Articles, Book Chapters and Other Papers

Infants with single ventricle require staged cardiac surgery, with stage I typically performed shortly after birth, stage II at 4 to 6 months of age, and stage III at 3 to 5 years of age. There is a high risk of interstage mortality and morbidity after infants are discharged from the hospital between stages I and II. Traditional home monitoring requires caregivers to record measurements of weight and oxygen saturation into a binder and requires families to assume a surveillance role. We have developed a tablet PC-based solution that provides secure and nearly instantaneous transfer of patient information to a …

A Comparison Of Right Ventricular Volumes In Children And Adults With Repaired Tetralogy Of Fallot - Including And Excluding Right Ventricular Trabeculations, James D. Enos, Jacob Hartz, Rachel Quinn, Lasya Gaur, Russell Cross, Laura J. Olivieri

Pediatrics Posters and Presentations

No abstract provided.

Effects Of Percutaneous Closure Of Patent Ductus Arteriosus In Infants Less Than One Year Of Age: A Systematic Review, Megan K. Locke

Undergraduate Honors Thesis Projects

The purpose of this study is to assess the safety and feasibility of percutaneous PDA closure in infants as reported in the available medical literature. Patent ductus arteriosus (PDA) is the continuous opening of the ductus arteriosus in an infant’s heart after birth. This, when left untreated, can lead to severe health complications. Historically, the first treatment options to correct PDA were the use of medications and surgical ligation. Percutaneous PDA closure is a newer treatment option that is increasingly performed during infancy (age); however, its safety and feasibility is not yet understood in infants. We conducted a systematic review …