Cardiology Commons^™

Systemic Arterial Function After Multisystem Inflammatory Syndrome In Children Associated With Covid-19, Ketaki Mukhopadhyay, Marla S. Johnston, James S. Krulisky, Shengping Yang, Thomas R. Kimball

School of Medicine Faculty Publications

Introduction: Multisystem inflammatory syndrome in children (MIS-C) is a new disease entity occurring in the pediatric population two to six weeks after coronavirus exposure due to a systemic arteritis. We investigated post-hospital-discharge arterial function at short- and mid-term intervals using pulse wave velocity. We assessed associations between arterial function, left ventricular diastolic and systolic function and left ventricular mass. Materials and methods: Retrospective data collection was carried out on 28 patients with MIS-C with at least two outpatient pediatric cardiology clinic visits post hospital admission. The patients underwent assessment of systemic arterial function and cardiac function. Data included pulse wave …

Post-Operative Diagnosis Of Anomalous Left Coronary Artery From The Right Pulmonary Artery Via Transthoracic Echocardiogram, Lavina Desai, Sanket Shah, Edo Bedzra, Christopher Mathis

Posters

A term infant female was diagnosed with a ventricular septal defect (VSD) and aortic coarctation. Preoperative echocardiography and computed tomography angiography (CTA) showed a normal right coronary artery origin, but inconclusive left coronary artery (LCA) origin. There was a normal flow profile in the LCA by echocardiography. She underwent repair with extended end-to-end anastomosis and VSD closure during which surgical inspection revealed a usual appearing, retro pulmonary path of the LCA towards the left sinus of Valsalva. Postoperative echocardiogram revealed mild global dysfunction but no regional wall motion abnormalities.   Repeat echocardiogram revealed severe dysfunction most prominent in the anterolateral and …

Anomalous Left Coronary Artery From The Noncoronary Sinus, Wesam Sourour, Jesus C. Jaile, Stefanie Cheang, Jacquelyn D. Brady, Timothy Pettitt

School of Medicine Faculty Publications

No abstract provided.

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 …

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 …

Postnatal Brain Trajectories And Maternal Intelligence Predict Childhood Outcomes In Complex Chd, Vincent K Lee, Rafael Ceschin, William T Reynolds, Benjamin Meyers, Julia Wallace, Douglas Landsittel, Heather M Joseph, Daryaneh Badaly, J William Gaynor, Daniel Licht, Nathaniel H Greene, Ken M Brady, Jill V Hunter, Zili D Chu, Elisabeth A Wilde, R Blaine Easley, Dean Andropoulos, Ashok Panigrahy

Faculty, Staff and Student Publications

Objective: To determine whether early structural brain trajectories predict early childhood neurodevelopmental deficits in complex CHD patients and to assess relative cumulative risk profiles of clinical, genetic, and demographic risk factors across early development.

Study Design: Term neonates with complex CHDs were recruited at Texas Children’s Hospital from 2005–2011. Ninety-five participants underwent three structural MRI scans and three neurodevelopmental assessments. Brain region volumes and white matter tract fractional anisotropy and radial diffusivity were used to calculate trajectories: perioperative, postsurgical, and overall. Gross cognitive, language, and visuo-motor outcomes were assessed with the Bayley Scales of Infant and Toddler Development and …

How We Heal Little Hearts: The Piccolo Procedure Explained, Rania Habib, Toby A. Rockefeller

Transformational Pediatrics Podcast

The Piccolo device is an innovative and minimally invasive way to close the Patent ductus arteriosus (PDA) in pediatrics. Dr. Rockefeller talks about how the Piccolo came to fruition, the benefits, and what else is on the horizon for the PDA closure world.

Congenital Disseminated Malignant Rhabdoid Tumor Mimicking A Vascular Lesion, Scott K Wang, Prabhath Mannam, Katerina Dukleska, Fabiola Balarezo

Faculty, Staff and Student Publications

A congenital disseminated malignant rhabdoid tumor (MRT) is an exceedingly rare and aggressive pediatric cancer marked by the presence of malignant rhabdoid cells in various organs, including the brain, kidneys, and soft tissues, at birth. It is often detected prenatally or shortly post-birth. The malignancy's aggressiveness results in a bleak prognosis, offering limited treatment options and low survival rates. Early diagnosis and comprehensive medical intervention are crucial, but managing this condition is complicated by its rarity. We herein presented a case of a 37 and 1/7 week gestation male infant with a rapidly growing arm soft tissue mass within two …

The Utility Of Interventional Anesthesia, Kylie Daguio, Kiley Jackson, Samantha Boever, Andrew Chang, James Keane, Leonard B. Goldstein

SOMA Faculty Publications

no abstract

Relationship Between Obesity And Global Longitudinal Strain In The Pediatric Single Ventricle Fontan Population Across Ventricular Morphologies., Nitin Madan, Doaa Aly, Melanie Kathol, Amulya Buddhavarapu, Thomas Rieth, Ashley K. Sherman, Daniel Forsha

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Obesity is associated with diminished myocardial function as measured by strain echocardiography in children and young adults with normal cardiac anatomy. Data are lacking about the effect of obesity on myocardial strain in patients with a single ventricle. In this study, the relationship between body mass index (BMI) and single ventricle myocardial strain in the Fontan population was assessed.

METHODS AND RESULTS: Thirty-eight abnormal BMI Fontan cases (21 overweight and 17 obese) and 30 normal BMI Fontan controls matched based on single ventricular morphology, age, and sex were included in the study. Ventricular morphology was categorized as single right …

Prenatal Diagnosis Of A Ductal-Dependent Branch Pulmonary Artery: Extra Vessels In The 3-Vessel And Trachea View., Anmol Goyal, Maria Kiaffas, Tara Swanson, Melanie Kathol, Sanket Shah, Nitin Madan

Manuscripts, Articles, Book Chapters and Other Papers

Obtaining a three-vessel (3V) and three-vessel and trachea (3VT) view from the fetal upper mediastinum is now considered to be part of standard imaging protocol for routine obstetric cardiac screening examinations. We report two fetal cases of an anomalous pulmonary artery origin, utilizing the standard 3V and 3VT views. Further imaging led to a rare diagnosis of bilateral ductus arteriosus with discontinuous branch pulmonary arteries in the absence of any other congenital heart defect. We briefly discuss the imaging features, differential diagnoses, and management of this rare entity.

Advancing Virtual At-Home Care For Community Health Center Patients Using Patient Self-Care Tools, Technology, And Education [Letter], Cheryl Modica, Joy H. Lewis, Curtis Bay

SOMA Faculty Publications

Abstract

Introduction

Health centers are community-based, patient directed primary care providers that offer accessible, high-quality primary care within medically underserved communities. Screening for cancer and managing complex chronic conditions such as diabetes, hypertension, obesity, and depression are vital services for the vulnerable populations seen by community health centers. Delivering care for complex chronic conditions and preventive services using virtual models that integrate self-care tools and technology is an important approach to increasing access for hard-to-reach patients served by health centers.

Objective

This study aimed to explore the use of a virtual care model, applied using a systems approach and patient-driven …

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

Posters

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 …

Transposition Of The Great Arteries With Intramural Left Main Coronary Artery: Salient Imaging Findings And Description Of Two Operative Techniques, Joshua Holbert, Manasa Gadiraju, Sanket Shah, Edo Bedzra

Posters

Background: Transposition of the great arteries (TGA) is a common cyanotic critical congenital heart disease, with a feasible prenatal diagnosis. Arterial switch operation (ASO) with/without VSD closure is the preferred surgical approach with added challenge when an intramural coronary artery (IMC) is present. ASO is technically quite demanding with IMC, and incidence of post-operative complications and mortality are potentially higher. We present our recent TGA-IMC experience focusing on salient features identified on echocardiography, CT and invasive angiograms, as well as variations in ASO surgical techniques for repair. Methods: Our echo imaging acquisition required high frequency transducers and shallow image depth …

Health Equity And Policy Considerations For Pediatric And Adult Congenital Heart Disease Care Among Minoritized Populations In The United States., Keila N. Lopez, Kiona Y. Allen, Carissa M. Baker-Smith, Katia Bravo-Jaimes, Joseph Burns, Bianca Cherestal, Jason F. Deen, Brittany K. Hills, Jennifer H. Huang, Ramiro W. Lizano Santamaria, Carlos A. Lodeiro, Valentina Melo, Jasmine S. Moreno, Flora Nuñez Gallegos, Harris Onugha, Tony A. Pastor, Michelle C. Wallace, Deidra A. Ansah

Manuscripts, Articles, Book Chapters and Other Papers

Achieving health equity in populations with congenital heart disease (CHD) requires recognizing existing disparities throughout the lifespan that negatively and disproportionately impact specific groups of individuals. These disparities occur at individual, institutional, or system levels and often result in increased morbidity and mortality for marginalized or racially minoritized populations (population subgroups (e.g., ethnic, racial, social, religious) with differential power compared to those deemed to hold the majority power in the population). Creating actionable strategies and solutions to address these health disparities in patients with CHD requires critically examining multilevel factors and health policies that continue to drive health inequities, including …

Development Of Right Ventricular Electromechanical Dyssynchrony Following Surgical Repair Of Tetralogy Of Fallot In Infants., Andrew W. Mccrary, Sydney D. Collins, Zebulon Z. Spector, P Andrea Kropf, Piers C A Barker, Joseph Kisslo, Daniel Forsha

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: In adolescents and adults with tetralogy of Fallot (TOF), right ventricle (RV) electromechanical dyssynchrony (EMD) due to right bundle branch block (RBBB) is associated with reduced exercise capacity and RV dysfunction. While the development of RBBB following surgical repair of tetralogy of Fallot (rTOF) is a frequent sequela, it is not known whether EMD is present in every patient immediately following rTOF. The specific timing of the onset of RBBB following rTOF therefore provides an opportunity to assess whether acute RBBB is associated with the simultaneous acquisition of EMD.

METHODS: Transthoracic echocardiography with speckle tracking analysis for RV global …

Maternal Western Diet Programs Cardiometabolic Dysfunction And Hypothalamic Inflammation Via Epigenetic Mechanisms Predominantly In The Male Offspring, Mona Elgazzaz, Clara Berdasco, Jone Garai, Melody Baddoo, Shiping Lu, Hisham Daoud, Jovanny Zabaleta, Franck Mauvais-Jarvis, Eric Lazartigues

School of Medicine Faculty Publications

Objective; Maternal exposure during pregnancy is a strong determinant of offspring health outcomes. Such exposure induces changes in the offspring epigenome resulting in gene expression and functional changes. In this study, we investigated the effect of maternal Western hypercaloric diet (HCD) programming during the perinatal period on neuronal plasticity and cardiometabolic health in adult offspring. Methods; C57BL/6J dams were fed HCD for 1 month prior to mating with regular diet (RD) sires and kept on the same diet throughout pregnancy and lactation. At weaning, offspring were maintained on either HCD or RD for 3 months resulting in 4 treatment groups …

Aortic Valve Leaflets Are Asymmetric And Correlated With The Origin Of Coronary Arteries, Taylor S Koerner, Thomas Cunningham, Mayme E Marshall, Lauren S Talley, Megan Childress, Rami M Kharouf, Wen Li, Jorge D Salazar, Antonio F Corno

Faculty, Staff and Student Publications

BACKGROUND: Asymmetry of the aortic valve leaflets has been known since Leonardo Da Vinci, but the relationship between size and shape and origin of the coronary arteries has never been examined. Our aim was to evaluate this anatomy in a population of pediatric patients using a cross-sectional study design.

METHODS: Consecutive pediatric patients with trans-esophageal echocardiography (TEE), with or without trans-thoracic echocardiography (TTE), were included in our study. Exclusion criteria: (I) bicuspid aortic valve; (II) aortic valve stenosis; (III) hypoplasia of aortic valve annulus, or aortic root; (IV) truncal valve; (V) coronary artery atresia; (VI) previous surgery on aortic valve …

Ablation Of Accessory Pathway From Right Atrial Appendage To Anatomic Left Ventricle In L-Transposition Of The Great Arteries, Shree Lata Radhakrishnan, Robert O. Drutel, Cody M. Williams, Raman Danrad, Kelly K. Gajewski, Paul A. Lelorier

School of Medicine Faculty Publications

No abstract provided.

Association Between Remote Monitoring And Interstage Morbidity And Death In Patients With Single-Ventricle Heart Disease Across Socioeconomic Groups., Bianca Cherestal, Lori A. Erickson, Janelle R. Noel-Macdonnell Phd, Girish S. Shirali, Hayley S. Hancock, Doaa Aly, Matthew Files, Sarah Clauss, Natalie Jayaram

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Despite improvements in survival over time, the mortality rate for infants with single-ventricle heart disease remains high. Infants of low socioeconomic status (SES) are particularly vulnerable. We sought to determine whether use of a novel remote monitoring program, the Cardiac High Acuity Monitoring Program, mitigates differences in outcomes by SES.

METHODS AND RESULTS: Within the Cardiac High Acuity Monitoring Program, we identified 610 infants across 11 centers from 2014 to 2021. All enrolled families had access to a mobile application allowing for near-instantaneous transfer of patient information to the care team. Patients were divided into SES tertiles on the …

Recognizing Postural Orthostatic Tachycardia Syndrome In Primary Care, Brittney Hulsey

PA Faculty Publications

Postural orthostatic tachycardia syndrome (POTS) is a complex autonomic disorder characterized by abnormal tachycardia on standing. This disorder predominantly affects young women, with a peak incidence between ages 20 and 30 years. POTS often is associated with a variety of symptoms, including dizziness, palpitations, fatigue, generalized weakness, anxiety, and exercise intolerance, which can significantly impair patient quality of life. Primary care providers (PCPs) often are the first healthcare professionals to whom patients present. This article provides an overview of POTS, including diagnostic criteria and the importance of a thorough clinical evaluation to rule out other causes of symptoms. PCPs play …

Temporal Artery Pseudoaneurysm In A Pediatric Patient, Gowri Gowda, Donald Mendoza, Amanda Tullos, Jessica Zagory, Malachi Sheahan

School of Medicine Faculty Publications

Superficial temporal artery pseudoaneurysms are an uncommon vascular pathology that can present after head or facial trauma. Furthermore, they are rarely reported in the pediatric population. Ultrasound can be a useful tool in the diagnosis because it is easily accessible and can be rapidly acquired. We report a case that demonstrates the utility of ultrasound in the diagnosis of a superficial temporal artery pseudoaneurysm.

Atrial Appendage Accessory Pathway Ablation: Tips For Recognition And Approach To Ablation, Nikola Dragisic, Emily R. Backes, Lindsey Malloy-Walton, Philip M. Chang

Manuscripts, Articles, Book Chapters and Other Papers

No abstract provided.

Left Juxtaposition Of The Right Atrial Appendage: Pitfalls In Diagnosis, Kanya Singhapakdi, Wesam Sourour, Thomas Kimball

School of Medicine Faculty Publications

Several congenital anomalies of the right atrial appendage (RAA) have been described including aneurysm, herniation (in association with a pericardial defect), and left juxtaposition. The left juxtaposition of the RAA (LJRAA), first described by Birmingham in 1893 and subsequently introduced by Dixon in 1954, is usually associated with complex cardiac malformations such as obstruction of the left ventricular outflow tract. In this case report, we will describe an unusual variant of LJRAA in the absence of any other cardiac defects, which was initially misinterpreted as an aortic dissection. The correct diagnosis was made after careful reinterpretation and the use of …

Noninvasive Point Of Care Device For Assessing Cardiac Response To Acute Volume Changes, Harry A. Silber, Nisha A. Gilotra, Thomas L. Miller

Department of Pediatrics Faculty Papers

Purpose: The change in the amplitude of a peripheral pulse in response to a Valsalva maneuver has diagnostic utility for assessing volume status at the bedside. We have developed a device to automatically quantify the Valsalva pulse response (VPR) to a standardized Valsalva maneuver that the device guides a user to perform. In this study, we sought to determine whether VPR by the device, Indicor, is sensitive enough to detect the acute increase in central pressure and volume load that occurs with a passive leg raise (PLR) in healthy volunteers.

Methods: Healthy volunteers were tested semirecumbently at 45 degrees, then …

Left Pulmonary Artery Occlusion Following Device Closure Of Patent Ductus Arteriosus In Premature Infants, Jesus C. Jaile, Ernest Siwik, Sergio Bartakian

School of Medicine Faculty Publications

Background: Device closure of a patent ductus arteriosus (PDA) is rapidly evolving, with the Amplatzer Piccolo Occluder (Abbott) receiving US Food and Drug Administration approval and becoming the first device approved for PDA closure in patients ≥700 g. We report on the first known cases of complete left pulmonary artery (LPA) occlusion following Piccolo closure of a PDA in premature infants. Methods: Retrospective chart analysis of PDA closures. Results: We have performed over 50 cases of Piccolo device closure of the PDA in preterm neonates in the past 2 years, with these 2 cases representing our only complications (4%). This …

3-Year Follow-Up Of A Prospective, Multicenter Study Of The Amplatzer Piccolo™ Occluder For Transcatheter Patent Ductus Arteriosus Closure In Children ≥ 700 Grams., Brian H. Morray, Shyam K. Sathanandam, Thomas Forbes, Matthew Gillespie, Darren Berman, Aimee K. Armstrong, Shabana Shahanavaz, Thomas Jones, Toby A. Rockefeller, Henri Justino, David Nykanen, Courtney Weiler, Dan Gutfinger, Evan M. Zahn

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVE: This study describes 3-year follow-up of 200 infants weighing ≥ 700 grams who underwent transcatheter patent ductus arteriosus (PDA) closure with the Amplatzer Piccolo™ Occluder.

STUDY DESIGN: Between June 2017 and February 2019, 200 children were enrolled in this U.S. study (NCT03055858). PDA closure, survival, and device- or procedure-related events were evaluated. A total of 156 of the available 182 patients (86%) completed the study.

RESULTS: The implant success rate was 95.5% (191/200). At 3 years, PDA closure was observed in 100% (33/33) of patients. Survival was >95% with 9 reported deaths. No deaths were adjudicated as device- or …

Cardiac Tamponade: Innovative Sternotomy Simulation Model For Training Pediatric Cardiac Intensive Care Team, Tolu Akinpelu, Mohammed Alhendy, Malarvizhi Thangavelu, Karen Weaver, Nicole Muller, James Mcelroy, Daniel Nento, Utpal Bhalala

Research Symposium

Introduction: Cardiac tamponade occurring after cardiac surgery is rare but life threatening and requires simultaneous resuscitation and emergent sternotomy by the intensive care team. A simulated scenario using innovative mannequin with sternotomy wound has the capability of reproducing cardiac arrest associated with postoperative tamponade. We evaluated the face validity of this innovative mannequin, the confidence level and crisis resource management skills of the team during sternotomy to manage postoperative cardiac tamponade.

Methods: The simulation case scenario was developed using innovative sternotomy mannequin for children’s hospital cardiac intensive care unit (CICU) teams. The case involved a 3-year old male, intubated, mechanically …

Congenital Complete Heart Block Complicated By Atrial Flutter Diagnosis And Management, Ali A. Alakhfash, Abdulrahman Almesned, Abdullah Alqwaiee, Hashem D. Alqurashi, Waleed Almanea, Mesned Abdulrahman

Journal of the Saudi Heart Association

Seventeen-month-old child was diagnosed in utero to have congenital complete heart block. The mother has Sjogren’s syndrome with high Anti Ro antibodies. The baby was delivered at term with a heart rate of 55 to 60 beats per minute. Echocardiography revealed a structurally normal heart with a small atrial septal defect and moderate patent ductus arteriosus. At the age of 17 months, he developed atrial flutter which was aborted using electrical cardioversion in the Cath lab. No recurrence of the atrial flutter during a one-year follow-up.

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

Posters

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 …