Pediatric Cardiology - UT Southwestern

Pediatric Cardiology
2011 Academic Review
The Division of Pediatric Cardiology is committed to:
• advancing scientific discovery through clinical and basic research,
• the provision of outstanding pediatric cardiac care,
• and the education of new physicians as well as the ongoing support of
practicing physicians throughout the region.
Research is strongly supported by UT Southwestern’s main campus resources,
including extensive core facilities for genetic evaluation and both surgical and
catheterization suites for large animal studies. Clinical research is further
augmented by the Department of Clinical Sciences, with support services for
study design, grant preparation and statistical analysis.
The clinical service benefits from a large referral base, including those from
regional, national and international sources in addition to the local metroplex
population. In collaboration with Children’s Medical Center, construction of
a new Heart Center is under way to bring all cardiac services together on one
floor of the hospital. A dedicated outreach program facilitates patient care.
The division actively participates in the training of students, residents and subspecialty fellows, which is provided by a
diverse and talented cardiology faculty, who themselves represent all of the major training programs in the United States.
Faculty
William Scott, M.D.
Professor,
Director, Pediatric Cardiology
The Division has 22 board-certified pediatric cardiologists, each with a special area of expertise,
including critical care medicine, inteventional catheterization, electrophysiology, advance
imaging, fetal cardiology, cardiac transplantation, heart failure, prevention and adults with
congenital heart disease.
In 2011, Dr. Bibhuti Das joined us from the University of Louisville where he directed the heart
transplant program. He will be providing expertise in the management of patients with heart
failure and with the heart transplantation program.
Dr. Tejas Shah moved to the University of Wisconsin to pursue his interests in transplantation
and adults with congenital heart disease.
Bibhuti Das, M.D.
Aliessa Barnes, M.D.
Assistant Professor
Shannon Blalock, M.D.
Assistant Professor
Sarah Blumenschein, M.D.
Clinical Associate Professor
Mark A. Clay, M.D.
Assistant Professor
Michael Day, M.D.
Assistant Professor
Bibhuti B. Das, M.D.
Associate Professor
Vivian Dimas, M.D.
Assistant Professor
David Fixler, M.D.
Professor
Candace Gibbin, M.D.
Assistant Professor
Lisa Heistein, M.D.
Assistant Professor
Catherine Ikemba, M.D.
Assistant Professor
Amy Juraszek, M.D.
Associate Professor, Pediatrics and
Pathology
Colin E. Kane, M.D.
Assistant Professor
Matthew Lemler, M.D.
Professor
Lynn Mahony, M.D.
Professor
Alan Nugent, M.D.
Associate Professor
Claudio Ramaciotti, M.D.
Professor
William Scott, M.D.
Professor, Division Director
Poonam Punjwani Thankavel, M.D.
Assistant Professor
Surendranath Veeram Reddy, M.D.
Assistant Professor
Shawyntee M. Vertilus, M.D., M.P.H.
Assistant Professor
Thomas Zellers, M.D.
Professor, Chief Medical Officer, CMC
Ilana Zeltser, M.D.
Assistant Professor
Additional faculty recruitments to support expansion in the areas of cardiac transplantation, heart failure, critical care,
prevention and advanced noninvasive imaging are ongoing.
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Research Activities
Basic Science
An exciting ongoing area of investigation is the development of biodegradable stents for use in the cardiovascular system.
Current research is directed at an evaluation of the tissue response to these materials in animals Another new area of
investigation is to optimize cardiovascular support therapy for pediatric patients with end stage heart failure. Currently the
only ventricular assist devices available require surgical placement. We are investigating an animal model for the feasibility
of a pediatric sized catheter delivered device. Animal research is also ongoing to optimize catheter-based interventions in
the fetus.
Clinical Sciences
The cardiology division participates in multiple multicenter trials. In interventional catheterization, current trials are
assessing various devices, including the Amplatzer and Helex occluders, and management of coarctation, including the
placement of stents. A multicenter effort is also underway to evaluate the use of ventricular assist devices in the pediatric
population.
In cardiac transplant medicine there are both multi-institutional trials and local investigative efforts to evaluate ABO
incompatibility, drug therapies, markers for cardiac transplant rejection and the role of pulmonary hypertension.
It is anticipated that a recently completed multicenter trial of athletic screening processes will be used for a broader national
evaluation. We are contributing to a quality initiative to assess the care of patients with hypoplastic left heart syndrome.
Other clinical investigations are underway to study coronary artery imaging, tricuspid valve disorders, racial and ethnic
differences in mortality for children with congenital heart disease, natural history studies of patent ductus arteriosus
in preterm neonates, evaluation of renal function following heart catheterization, innovations in noninvasive cardiac
monitoring and the psychological impact of prenatal diagnosis of congenital heart disease.
Collaborative studies within the department of Pediatrics include protocols to evaluate stroke, migraine, muscular
dystrophy, neonatal asphyxia, and complications of sickle cell disease and childhood cancer.
Clinical Activities
Pediatric Cardiology faculty provided more than 8,000 inpatient, outpatient and diagnostic services in 2011. Inpatients with
cardiac disease received care in the 20-bed CVICU and a 22-bed telemetry/step-down unit. Last year, care was provided for
523 surgical patients, including 336 open cases and nine cardiac transplants. The average length of stay is 12.05 days in the
CVICU and 7.03 days on the cardiology inpatient unit.
Nearly 10,734 patients were evaluated in the outpatient clinics at Children’s main campus, Legacy and Southlake facilities.
In addition to overall cardiac evaluation, specialty clinics provided focused care for patients with implanted devices,
arrhythmia, obesity, cholesterol and lipid disorders, heart transplantation, Marfan syndrome and adults with congenital
heart disease.
Diagnostic services included 856 cardiac catheterizations, most involving interventional procedures. Eighty-six of those
were electrophysiologic procedures including catheter ablation. Noninvasive studies are comprised of imaging and
electrocardiographic (ECHO) studies. The division performed:
• 6,616 transthoracic ECHOs,
• 632 transesophageal studies,
• and 279 fetal ECHOs.
Additional studies included:
• 14,058 electrocardiograms (ECGs),
• 797 Holter monitors,
• 155 pacemaker analyses,
• 164 exercise tests,
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• and 87 cardiac MRIs.
Additionally, telemedicine capabilities for transmission and review of echocardiographic (ECHO) and electrocardiographic
(ECG) studies are available to multiple institutions across the state, from Amarillo to Tyler. Approximately 549 ECHOs and
100 ECGs were interpreted from these institutions.
Education and Training
The Division provided educational opportunities for medical
students and pediatric residents, including third-year pediatric
rotations and fourth-year electives in pediatric cardiology.
The fully-accredited fellowship program offers comprehensive
training in all aspects of pediatric cardiology. Two fellows are
accepted each year. Advanced fellowships are available in critical care,
interventional catheterization, cardiac imaging, transplantation and
electrophysiology. Currently there are six fellows in the categorical
program and two receiving advanced training.
Two fellows graduated in 2011:
• Adrian Dyer, M.D., stayed at UT Southwestern to complete a
fourth-year fellowship in cardiac imaging.
• Jason Imundo, M.D., went to Children’s Hospital of Philadelphia
to complete a fourth-year fellowship in electrophysiology.
Presentations
Dr. Adrian Dyer
Poster presentation at the 2 nd Annual
Pediatric Senior Fellow Research Day
Aliessa Barnes, M.D.
• “Donors’ Characteristics and Impact on Outcomes in Pediatric Heart Transplant Recipients” and “Use of Bortezomib
in Management of Humoral Rejection in a Non-Compliant Teenage Heart Transplant,” International Society for Heart
and Lung Transplantation’s 31 st Annual Meeting and Scientific Sessions, San Diego, CA, April 2011
• “Management of Single Ventricle Patients with Berlin Heart EXCOR Ventricular Assist Device: Single Center
Experience,” International Society for Pediatric Mechanical Cardiopulmonary Support, Philadelphia, PA, May 2011
Shannon Blalock, M.D.
• “MRI and Pulmonary Hypertension,” ACC.11 Session: Beyond Eisenmenger - Pulmonary Hypertension in Pediatrics,”
Invited Lecture, American College of Cardiology Annual Meeting, New Orleans, LA, April 2011
• Blalock SE, Ogawa M, Chan F, Maxey D, Rosenthal DN, Feinstein JA. “MRI Evaluation of the Right Ventricle in
Pediatric Pulmonary Hypertension,” International Society of Heart Lung Transplantation Annual Conference, San
Diego, CA, April 2011 (Oral abstract presentation)
Sarah Blumenschein, M.D.
• “Hypercholesterolemia in Children,” National Association of Pediatric Nurse Associates and Practitioners, Arlington,
TX, October 2011
Bibhuti Das, M.D.
• “Outcome of Fetal Listing for Heart Transplantation,” American Heart Association Annual Conference, Orlando FL,
November 2011
Amy Juraszek, M.D.
• “The Surgical Pathology of Transposition,” Annual Pediatric Cardiac Care Conference, Austin, TX, February 2011
• “Small Group Demonstrations of Congenital Heart Disease Pathology,” 17 th Annual Pediatric Echocardiography
Conference, Atlanta, GA, April 2011
• “Pre-sports Participation Evaluation,” 43 rd Annual Kenneth C. Haltalin Conference Pediatric for the Practitioner,
Dallas, TX, April 2011
• “Introduction to Cardiac Anatomy and Nomenclature,” “ASD, VSD, CAVC,” “Left Heart Obstructions,” “Conotruncal
Malformations” and “Heterotaxy and Anatomically Single Ventricles,” The Heart Center at Nationwide Columbus
Children’s Hospital, Columbus, OH, October 2011
William Scott, M.D.
• “Duchenne Muscular Dystrophy: Optimizing Cardiac Care,” DMD, and Pediatric Neuromuscular Diseases, Texas
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Scottish Rite Hospital for Children, Dallas, February 2011
• “Risk Stratification for Sudden Cardiac Death in Children: What Do We Really Know and When Do We Know It?,”
Pediatric Electrophysiology Symposium, Boston, MA, October 2011
Poonam Punjwani Thankavel, M.D.
• “Echocardiographic Prediction of Spontaneous Closure of the Ductus Arteriosus in Neonates Less Than 30 Weeks of
Gestation,” Pediatric Academic Societies, Denver, CO, February 2011
• “Double Switch with LVOT Resection and Mitral Valve Repair,” Congenital Heart Surgeons’ Society’s Annual
Meeting, Chicago, IL, October 2011
• “Repair of DORV with Subpulmonary VSD, D-TGA and Type B Aortic Arch Interruption,” Southern Thoracic
Surgical Association’s 58 th Annual Meeting, San Antonio, TX, November 2011
Awards and Honors
Sarah Blumenschein, M.D.
• Texas Super Doctor , Texas Montly
Michael Day, M.D.
• Best Pediatric Specialists in Dallas, D Magazine
David Fixler, M.D.
• Best Pediatric Specialists in Dallas, D Magazine
Matthew Lemler, M.D.
• Top US Doctors, US News & World Report
Lynn Mahony, M.D.
• Texas Super Doctor , Texas Montly
• Top US Doctors, US News & World Report
• National Heart, Lung, and Blood Institute (NHLBI) Leadership
Appreciation Award
Alan Nugent, M.D.
• Best Pediatric Specialists in Dallas, D Magazine
Claudio Ramaciotti, M.D.
• Texas Super Doctor, Texas Montly
William Scott, M.D.
• Best Doctors in America
• Best Pediatric Specialists in Dallas, D Magazine
• Texas Super Doctor, Texas Montly
Thomas Zellers, M.D.
• Best Pediatric Specialists in Dallas, D Magazine
• Texas Super Doctor, Texas Montly
Publications
Dr. Jason Imundo
Oral presentation at the 2 nd Annual
Pediatric Senior Fellow Research Day
1. Artman M, Mahony L, Teitel D. Neonatal Cardiology, McGraw Hill Inc., New York, NY, 2nd edition, 2011
2. Atz AM, Travison TG, McCrindle BW, Mahony L, et al. Late status of Fontan patients with persistent surgical
fenestration. J Am Coll Cardiol;57:2437-43.
3. Atz AM, Zak V, Breitbart RE, Colan SD, Pasquali SK, Lu M, Mahony L, et al. Factors associated with serum brain
natriuretic peptide levels after the Fontan procedure. Congenit Heart Dis;6:313-21.
4. Balakrishnan PL, Guleserian KJ, Schuster J, Blalock SE. Congenital arteriovenous malformation between the
subclavian artery and superior vena cava presenting in neonatal heart failure. Pediatr Cardiol;32:1204-6.
5. Bergersen L, Gauvreau K, Justino H, Nugent A, et al. Randomized trial of cutting balloon compared with highpressure
angioplasty for the treatment of resistant pulmonary artery stenosis. Circulation;124:2388-96.
6. Conway J, Chin C, Kemna M, Birch M, Barnes A, et al. Donors characteristics and impact on outcomes in pediatric
heart transplant recipients. J Heart & Lung Transplantation 2011;30:S133.
7. Crary SE, Ramaciotti C, Buchanan GR. Prevalence of pulmonary hypertension in hereditary spherocytosis. Am J
Hematol;86:E73-6.
8. Das BB, Shoemaker L, Kim E, Mascio CE, Austin EH. Severe calcification of the aorta (porcelain aorta) associated with
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sarcoidosis in a pediatric heart transplant recipient. Pediatr Transplant.
9. Dowling MM, Ikemba CM. Intracardiac shunting and stroke in children: a systematic review. J Child Neurol;26:72-82.
10. Hubail Z, Lemler M, Ramaciotti C, Moore J, Ikemba C. Diagnosing a patent foramen ovale in children: is
transesophageal echocardiography necessary? Stroke;42:98-101.
11. Kane C, Kogon B, Pernetz M, et al. Left ventricular function improves after pulmonary valve replacement in patients
with previous right ventricular outflow tract reconstruction and biventricular dysfunction. Tex Heart Inst J;38:234-7.
12. Marino BS, Drotar D, Cassedy A, Davis R, Tomlinson RS, Mellion K, Mussatto K, Mahony L, et al. External validity of
the pediatric cardiac quality of life inventory. Qual Life Res;20:205-14.
13. Massimiliano C, Simona S, Bruno M, Aldo C, Das BB. Clinical relevance of different B-type natriuretic peptide decisional
cutoff values for the diagnosis of congenital heart disease in the first weeks of life. Pediatr Cardiol;32:537; author reply -8.
14. Mir A, Lemler M, Ramaciotti C, Blalock S, Ikemba C. Hypertrophic Cardiomyopathy in a Neonate Associated with
Nemaline Myopathy. Congenit Heart Dis; Nov 2011[Epub ahead of print]
15. Mir A, Obafemi A, Young A, Kane C. Myocardial infarction associated with use of the synthetic cannabinoid K2.
Pediatrics;128:e1622-7.
16. Nembhard WN, Salemi JL, Ethen MK, Fixler DE, Dimaggio A, Canfield MA. Racial/Ethnic disparities in risk of early
childhood mortality among children with congenital heart defects. Pediatrics;127:e1128-38.
17. Ravishankar C, Tabbutt S, Allen K, Cooper DS, Frank DU, Frommelt P, Ghanayem N, Goldberg C, Graham EM, Kirsh
JA, Krawczeski CD, Lai WW, Lewis A, Lodge AJ, Mahony L, et al. Risk Factors for Mortality in the Single Ventricle
Reconstruction Trial: Beyond Shunt Type. Circulation 2011;124:A9867.
18. Rehman M, Jenkins KJ, Juraszek AL, et al. A prospective phase II trial of vinblastine and methotrexate in multivessel
intraluminal pulmonary vein stenosis in infants and children. Congenit Heart Dis;6:608-23.
19. Shah T, Fixler D, Barnes A. Use of Bortezomoib in management of humoral rejection in a non-compliant teenage
heart transplant. . J Heart & Lung Transplantation 2011;26:S179-S80.
20. Thankavel PP, Ramaciotti C, Lemler MS. A neonate with left congenital diaphragmatic hernia and transient
retrograde flow in the aortic arch. Pediatr Cardiol;32:1207-8.
21. Veeram Reddy SR, Welch T, Wang J, Bernstein F, Richardson J, Eberhrt R, Forbess J, Nugent A, et al. A Novel
Biodegradable Stent Design Applicable for Pediatric Use in Congenital Heart Disease : Bench Testing and Initial
Results in a Rabbit Model. Circulation 2011;124:A16010.
22. Vogel M, McElhinney DB, Wilkins-Haug LE, Marshall AC, Benson CB, Juraszek AL, et al. Aortic stenosis and severe
mitral regurgitation in the fetus resulting in giant left atrium and hydrops: pathophysiology, outcomes, and preliminary
experience with pre-natal cardiac intervention. J Am Coll Cardiol;57:348-55.
23. Weintraub RG, Nugent AW, Davis A, King I, Barucha T, Daubeney PEF. Presentation, echocardiographic findings and
long term outcomes in children with familial dilated cardiomyopathy. Prog Pedatr Cardiol 2011;31:119-22.
24. Young AC, Schwarz E, Medina G, Obafemi A, Feng SY, Kane C, et al. Cardiotoxicity associated with the synthetic
cannabinoid, K9, with laboratory confirmation. Am J Emerg Med.
Grants
NIH and Other Government Subcontracts
Lynn Mahony, M.D.
“Pediatric Heart Disease Clinical Research Network”
New England Research Inst./National Heart, Lung and Blood Institute, 5 U01 HL068270-11
09/01/11 - 06/30/12
Ilana Zeltser, M.D.
“Early Cardiovascular Screening Pilot (CVSP)”
Championship Hearts Foundation
01/15/08 – 08/31/11
Sponsored Clinical Trials
Aliessa Barnes, M.D.
Pharmacokinetics and Safety of Valganciclovir in Pediatric Heart Transplant Recipients < 4 Months of Age
Hoffmann-La Roche, Inc., NP22523
03/16/09 - 08/31/12
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Vivian Dimas, M.D.
Amplatzer Duct Occuluder II Clinical Study
AGA Medical Corporation, ADO II
01/27/09 – 08/31/12
Lynn Mahony, M.D.
“Quality of Life Assessment in Pediatric Cardiac Population: Testing the Pediatric Quality of Life Inventory”
Cincinnati Children’s Hospital, 22504-01-05
04/26/05 – 06/30/13
Thomas Zellers, M.D.
Post Market Surveillance Plan for the Amplatzer Duct Occluder
AGA Medical Corporation, IDE G980103
05/15/07 - Present
Closure of Muscular Ventricular Septal Defects with the AMPLATZER Muscular VSD Occluder -- Post Approval
Study
AGA Medical Corporation, AGA016
02/17/06 - Present
Comparison Between Surgical vs. Balloon Angioplasty vs. Intravascular Stent Placement for Recurrent or Native
Coarctataion of the Aorta
Children’s Hospital of Michigan, CCISC
04/16/07 - 08/31/11
GORE HELEX Septal Occluder: Post Approval Study
W. L. Gore & Associates Inc., HLX06-04
12/12/07 – Present
Multicenter Continued Access Study of the Helex Septal Occluder
W.L. Gore & Associates Inc., HLX0301
01/01/04 - Present
Coarctation of the Aorta Stent Trial (COAST)
Johns Hopkins University
05/01/08 - 08/31/12
Melody Transcatheter Pulmonary Valve Post Approval Study
Medtronic, Inc., Melody
01/05/11 - 12/31/12
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