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Page 1: Heart AR - texaschildrens.org

2014

Texas Children’s Heart Center

6621 Fannin Street

Houston, Texas 77030

texaschildrens.org/heartoutcomestexaschildrens.org/refer

©2015 Texas Children’s Hospital. All Rights Reserved. Heart212 061515

Page 2: Heart AR - texaschildrens.org

H E A R T C E N T E R 2 0 1 4 1

H E A R T C E N T E R O U T C O M E S 2 0 1 4

Table of Contents

Welcome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2

Texas Children’s Hospital and

Baylor College of Medicine . . . . . . . . . . . . . . . . . 3

Texas Children’s Heart Center . . . . . . . . . . . . . . 4

Congenital Heart Surgery Service . . . . . . . . . . . . 8

Coronary Anomalies Program . . . . . . . . . . . . . . 17

Heart Failure and Transplant Program . . . . . . . . 18

Interventional Cardiology . . . . . . . . . . . . . . . . . 24

Electrophysiology . . . . . . . . . . . . . . . . . . . . . . . . 27

Diagnostic Testing and Cardiac Imaging . . . . . . . 29

Adult Congenital Heart Disease Program . . . . 31

Cardiovascular Anesthesia . . . . . . . . . . . . . . . . . 33

Developmental Outcomes . . . . . . . . . . . . . . . . . 36

Cardiovascular Intensive Care Unit . . . . . . . . . . 38

Quality, Innovation and Research . . . . . . . . . . . . 40

Leadership Team . . . . . . . . . . . . . . . . . . . . . . . . . 44

Medical Staff Directory . . . . . . . . . . . . . . . . . . . 46

Publications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 48

Referrals . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 54

For access to Texas Children’s Heart Center outcomes

information, visit texaschildrens.org/heartoutcomes.

Page 3: Heart AR - texaschildrens.org

H E A R T C E N T E R 2 0 1 42

Dear colleagues, parents and friends:

We are pleased to present the 2014 Texas Children’s Heart Center Outcomes Book. Here you

will find highlights from our Heart Center programs and important data about patient outcomes

and innovative research programs.

We care for children of every age, and we are beginning to care for patients earlier than ever

before. Texas Children’s Heart Center works hand in hand with physicians in Texas Children’s Fetal

Center™ to identify and choose the best treatment plans for patients and their families. Surgery

completed while the fetus is still in the womb is a cutting-edge treatment that gives the patient

additional developmental time in the uterus, setting the stage for a healthier entry into the world.

Texas Children’s Hospital continues to have one of the largest pediatric solid organ transplant

programs in the country. In 2014, our team performed 95 transplants, including 32 heart

transplants, a record number for the hospital.

One of the joys of our work is seeing children thrive. In this year’s edition, we check in on two

congenital heart patients who have become part of our extended family. These young people

have not let heart disease hold them back from living full and active lives. As our patients

enter adulthood, many of them continue into our adult congenital heart program where our

cardiologists are experienced in treating patients with mended hearts.

Texas Children’s Heart Center strives to provide every procedure available for patients with

pediatric and congenital heart disease. Through our innovations in research and commitment to

improve all aspects of patient care, our goal is the very best possible treatment for every patient.

We hope you find this information useful. Thank you for your interest in our center.

Sincerely,

Charles D. Fraser, Jr., M.D.Surgeon-in-Chief, Texas Children’s HospitalDonovan Chair and Chief of Congenital Heart Surgery, Texas Children’s HospitalSusan V. Clayton Chair in Surgery, Baylor College of Medicine Professor of Surgery and Pediatrics, Baylor College of Medicine

Daniel J. Penny, M.D., Ph.D., M.H.A.Chief of Cardiology, Texas Children’s HospitalProfessor of Pediatrics, Baylor College of Medicine

Emad B. Mossad, M.D.Director of Pediatric Cardiovascular Anesthesiology, Texas Children’s HospitalProfessor of Anesthesiology and Pediatrics, Baylor College of Medicine

Lara S. Shekerdemian, M.D., F.R.A.C.P., M.H.A.Chief of Critical Care, Texas Children’s HospitalProfessor of Pediatrics, Baylor College of Medicine

Page 4: Heart AR - texaschildrens.org

H E A R T C E N T E R 2 0 1 4 3

Texas Children’s operates Texas Children’s Hospital, Texas Children’s Health Plan, the nation’s first

health maintenance organization (HMO) created just for children, and Texas Children’s Pediatrics,

the nation’s largest primary pediatric care network with 50 practices throughout the greater

Houston community.

Texas Children’s Hospital’s main campus is located near downtown Houston in the Texas Medical

Center, the largest medical center in the world. The main campus includes 650 inpatient beds, the

Clinical Care Center for outpatient visits, the Feigin Center for pediatric research and Texas

Children’s Pavilion for Women. To serve the rapidly growing population in West Houston, Texas

Children’s Hospital West Campus opened in 2011 as a community hospital and clinic and contains

ICU beds, inpatient beds, an emergency center, surgical suites and more than 20 subspecialty clinics.

In 2017, we will open our second community hospital, Texas Children’s Hospital The Woodlands.

Texas Children’s mission is to create a healthier future for children and women throughout our

global community by leading in patient care, education and research. Renowned worldwide for our

expertise and breakthrough developments in clinical care and research, Texas Children’s Hospital

was recently ranked #4 among top children’s hospitals in the nation and was ranked in all 10

pediatric subspecialties in U.S.News & World Report’s list of America’s Best Children’s Hospitals.

Texas Children’s Hospital is affiliated with Baylor College of Medicine in the areas of pediatrics,

pediatric surgery, and obstetrics and gynecology. Baylor is ranked by U.S.News & World Report as

one of the nation’s top 25 medical schools for research. Currently and throughout our 60-year

partnership, Texas Children’s Hospital serves as Baylor’s primary pediatric training site, and Baylor

faculty are the division chiefs and staff physicians of Texas Children’s patient care centers. The

collaboration between Texas Children’s Hospital and Baylor is one of the top 10 such partnerships

for pediatric research funding from the National Institutes of Health.

More than 1,500 board-certified pediatricians, pediatric subspecialists, pediatric surgical subspecialists

and dentists – offering the highest level of pediatric care in more than 40 subspecialties, programs

and services – practice at Texas Children’s with a support staff in excess of 10,500.

Texas Children’s Hospital and Baylor College of Medicine

Page 5: Heart AR - texaschildrens.org

H E A R T C E N T E R 2 0 1 44

Texas Children’s Heart Center is located in the hospital’s

main inpatient building, the West Tower, on floors 15 and

17 through 20. The close proximity of the Heart Center’s

treatment and administrative areas, perhaps unique in

pediatric cardiac care, enables team members to respond

quickly to patient and family needs or emergencies and

promotes collaboration among our multidisciplinary team.

Most recently, Texas Children’s Board of Trustees approved

expansion plans for the hospital’s main campus that

includes a 19-story addition to the top of an existing

building, adding 640,000 square feet and resulting in a

25-floor tower. Slated to be completed in 2018, the new

tower will house 130 beds for pediatric and cardiovascular

intensive care, new operating rooms with the latest

technology and will be the new home to Texas Children’s

Heart Center, including the outpatient clinic, cardiovascular

operating rooms and catheterization labs.

15

17181920

20 th FLOOR – Heart Center clinics and diagnostic facilities, including the echocardiography lab and cardiovascular clinical research core

19 th FLOOR – Heart Center administrative offices

and conference rooms

18 th FLOOR – 21-bed cardiovascular intensive care unit; dedicated Heart Center pharmacy; three

state-of-the-art cardiovascular operating rooms;

three state-of-the-art cardiac catheterization labs;

eight-bed holding/catheterization recovery unit

17th FLOOR – Cardiovascular anesthesiology

administrative offices; Ronald McDonald® Family Waiting Room with additional private rooms for

families to consult with their child’s physicians or to wait

during procedures; support services, including social

work and child life staff

15th FLOOR – 35-bed inpatient unit including six

“step-down” beds for patients who are healthy enough

to leave intensive care but still need close monitoring

Page 6: Heart AR - texaschildrens.org

H E A R T C E N T E R 2 0 1 4 5

Nº2

Pioneers in Pediatric Cardiac CareTexas Children’s Hospital has been a leader in pediatric heart care for more than 50 years. Led

by Denton Cooley, M.D., and Dan McNamara, M.D., the hospital started performing heart surgery

in children in 1956. Dr. McNamara, a patriarch of pediatric cardiology, and Dr. Cooley, a pioneer

heart surgeon, were among the first to demonstrate that small children could safely undergo

heart surgery.

Today, Texas Children’s Heart Center is one of the nation’s leading providers of pediatric cardiac

care. In 2014, U.S.News & World Report ranked Texas Children’s Heart Center #2 in the nation

for pediatric cardiology and heart surgery. Comprised of several multidisciplinary teams working

in conjunction with pediatric subspecialists throughout the hospital, the Heart Center provides

unparalleled care at every point from diagnosis through treatment and follow-up.

Texas Children’s Heart Center is part of the

OptumHealth Pediatric Transplant Centers of Excellence

Network and is recognized by Aetna as a Pediatric

Congenital Heart Surgery Institute of Excellence.

Page 7: Heart AR - texaschildrens.org

H E A R T C E N T E R 2 0 1 46

National and International ReachTexas Children’s commitment to caring for children reaches around the globe. Our Heart

Center cares for patients from over 15 countries around the world and more than 35 states

in the United States. We are dedicated to creating a healthier future for children throughout

the global community.

Page 8: Heart AR - texaschildrens.org

H E A R T C E N T E R 2 0 1 4 7

2014Cardiac catheterizations

Diagnostic testing and cardiac imaging

24,832

1,781

35,341

515

24

4,988

1,215

Surgical procedures

843

32

16

37

1,150TOTA L

68,696TOTA L

Echocardiograms

Fetal echocardiograms

Electrocardiograms

Cardiac MRIs

Stress MRIs

Holter® monitors

Stress tests

Cardiac operations

Heart transplants

Lung transplants

Adult congenital heart disease operations

Diagnostic cardiac catheterizations

Interventions

Electrophysiology studies and ablations

Pacemaker and defibrillator implants

503

446

141

60

928TOTA L

Cardiovascular anesthesia

1,094

1,002

152

430

Cardiovascular intensive care

616

420

2,678TOTA L

1,036TOTA L

Catheterizations and electrophysiology patients

Cardiovascular operating room patients

Interventional radiology patients

MRI patients

Surgical admissions

Medical admissions

2014 Texas Children’s Heart Center Overview

The outpatient clinic at Texas Children’s Heart Center had more than 23,000 patient

encounters in 2014.

Page 9: Heart AR - texaschildrens.org

H E A R T C E N T E R 2 0 1 48

Congenital Heart Surgery ServiceThe Congenital Heart Surgery Division provides individualized and comprehensive surgical care

for all aspects of pediatric and adult congenital heart disease. We are experienced in the rarest

of cases such as ectopia cordis and other infrequently seen conditions. Texas Children’s Heart

Center performs more than 900 surgical procedures annually with outcomes among the best in

the country.

We treat children of all ages, including preterm and low-birth-weight newborns, and we

personalize treatments and procedures to best suit the situation of each child and family. This

tailored approach includes cardiopulmonary bypass and neuroprotection strategies focused on the

patient’s condition and needs, helping to achieve optimal functional outcomes. The center’s Heart,

Lung and Heart-Lung Transplant Programs, among the largest and most successful in the United

States, are also part of the Congenital Heart Surgery Division.

Cardiovascular and thoracic surgery cases Volume by year

2014

928

2013

88

9

2 0 05

70

3

2 0 06

781

20 07

769

2 0 08

816

2010

83

6

2 0 09

911

2011

83

6

2012

767

Since 1995, Charles D. Fraser, Jr., M.D., and the Congenital Heart Team

have performed more than 13,600 operations on children and adults

with congenital heart disease.

According to the Society of Thoracic Surgeons National Database Program, congenital

heart surgery programs are considered high-volume programs if they perform more

than 250 index operations per year on average.

Page 10: Heart AR - texaschildrens.org

H E A R T C E N T E R 2 0 1 4 9

Total cardiopulmonary bypass operationsVolume by year

C P B N O N - C P B

2014

597

331

928

911

2 013

549

34

08

89

2 0 05

475

228

703

2 0 06

50

8273

781

2 0 07

48

0289

769

2 0 08

523

293

816

2 0 09

582

329

2 010

539

297

836

2 011

536

30

0836

2 012

483

28

4767

More than 64 percent of operations performed require the use of cardiopulmonary

bypass (CPB). Our incidence of mechanical circulatory support postcardiotomy is less

than 1 percent.

Our patients include children of all ages as well as adults who have been treated since

infancy or who have been diagnosed with a congenital heart defect later in life.

Cardiovascular and thoracic surgery cases Percent by age

N E O N AT E I N FA N T C H I L D A D U LT

2014

4.0

%4

4.5

%32

.9%

18.6

%

2013

5.1

%

47.4

%

27.7

%

19.9

%

2012

5.1

%

46

.3%

30

.4%

18

.3%

2010

8.3

%

46

.9%

28

.0%

16

.9%

2011

7.5

%

45

.3%

30

.6%

16

.5%

Page 11: Heart AR - texaschildrens.org

H E A R T C E N T E R 2 0 1 410

2014Mortalities by RACHS-1 classification in 2014

Total for Risk Category 1

Total for Risk Category 2

Total for Risk Category 3

Total for Risk Category 4

Total for Risk Category 5-6

Grand total

99

223

206

59

22

609

0

0

0

2

4

6

0.0%

0.0%

0.0%

3.4%

18.2%

<1.0%

0.3%

0.9%

3.6%

6.3%

15.0%

3.2%

Primaryprocedure

Number of

procedures

Number of

discharge

mortalities

%

mortality

STS

national

benchmark

<1.0%Overall risk-adjusted hospital mortality rate for our program in

2014 was less than 1.0%2. Data collected by the Society of Thoracic

Surgeons (STS) shows the national hospital discharge mortality rate

at 3.2%3.

1 Jenkins KJ, Gauvreau K, Newburger JW, et al., Consensus-based method for risk adjustment for surgery for congenital heart disease. J Thorac Cardiovasc Surg. 2002; 123:110-8.

2 007-RACHS-1 Index Surg CHD Volume.3 Society of Thoracic Surgeons Data Harvest Report, published May 2014.

The Risk Adjustment in Congenital Heart Surgery (RACHS-1)1 categorization is a

widely used risk stratification model that analyzes outcomes in congenital heart surgery.

The most common surgeries for congenital heart defects are stratified into six risk

categories. Surgeries with higher risk are placed in higher categories with Category 6

representing congenital heart surgeries associated with the greatest risk.

Page 12: Heart AR - texaschildrens.org

H E A R T C E N T E R 2 0 1 4 11

2014

Age-appropriate surgical planning and management allows for outcomes in patients of all

ages to be better than the national benchmarks. Patient grand total combines CPB and

non-CPB cases (N=609).

Mortalities by age and operation type in 2014

Neonate (0d - 30d)

Infant (31d - 1y)

Child (1y - 18y)

Adult (18y+)

Grand total

77

188

238

29

532

29

29

19

0

77

5

1

0

0

6

0

0

0

0

0

5/106

1/217

0/257

0/29

6/609

4.7%

0.5%

0.0%

0.0%

<1.0%

Primaryprocedure

CPB

cases

Non-CPB

cases

CPB

discharge

mortalities

Non-CPB

discharge

mortalities

Discharge

Mortality

9.0%

2.7%

1.1%

2.0%

3.2%

%

Mortality

STS

national

benchmark

Page 13: Heart AR - texaschildrens.org

H E A R T C E N T E R 2 0 1 412

Atrial septal defect repairVolume by year

2014

42

2013

35

2012

27

2006

23

2007

24

200814

2009

32

2010

14

2011

22

OV E R A L L H O S P ITA L

D I SC H A RG E

M O RTA L IT Y

R ATE 4

S T S N AT I O N A L

B E N C H M A R K 5 <1%

0%

The Congenital Heart Surgery Division performs an

average of 26 atrial septal defect (ASD) closures annually.

This includes patients with secundum, coronary, sinus

and sinus venosus ASDs. Our hospital survival rate is

100 percent. Our service also offers a minimally invasive

approach to ASD closure.

OV E R A L L H O S P ITA L

D I SC H A RG E

M O RTA L IT Y

R ATE 4

S T S N AT I O N A L

B E N C H M A R K 5 <1%

0%

Ventricular septal defect repairVolume by year

2014

57

2013

82

2012

56

20 06

47

20 07

53

20 0 8

59

2 0 09

53

2010

54

2011

60

Ventricular septal defects (VSD) are the most commonly

diagnosed congenital heart defects, and surgical treatment

remains the standard of care for children with this condition.

This data includes patients having perimembranous, supracristal,

inlet or muscular VSDs. They also include patients with

double-chambered right ventricle. The Congenital Heart

Surgery Division performs an average of 59 VSD closures

each year and in 2014 had a survival rate of 100 percent.

4 Hospital mortality is calculated over the last five years from 2010-2014.5 STS benchmark data is from 2010-2013.

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H E A R T C E N T E R 2 0 1 4 13

Atrioventricular canal repairVolume by year

2014

33

2013

20

2012

20

20 06

38

20 07

30

20 08

32

20 09

40

2010

31

2011

19

OV E R A L L H O S P ITA L

D I SC H A RG E

M O RTA L IT Y

R ATE 6

S T S N AT I O N A L

B E N C H M A R K 7 2.2%

.9%

Tetralogy of Fallot repairVolume by year

2014

24

2013

22

2012

27

20 06

42

20 07

21

20 08

27

20 09

35

2010

31

2011

30

OV E R A L L H O S P ITA L

D I SC H A RG E

M O RTA L IT Y

R ATE 6

S T S N AT I O N A L

B E N C H M A R K 7 1.6%

.9%

The Congenital Heart Surgery Division performs an average

of 28 operations each year for partial, intermediate and

complete atrioventricular (AV) canal defect.

Our surgical approach to the repair of tetralogy of Fallot

(TOF) is individualized for each patient based on age,

anatomy and symptoms. This allows us to repair the defect

and preserve critical parts of the cardiac anatomy, thereby

optimizing the patient’s cardiac function over the long

term. The Congenital Heart Surgery Division performs an

average of 27 TOF repairs each year.

6 Hospital mortality is calculated over the last five years from 2010-2014.7 STS benchmark data is from 2010-2013.

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H E A R T C E N T E R 2 0 1 414

Arterial switch operationVolume by year

2014

18

2013

19

2012

10

2011

14

20 06

29

20 07

16

20 08

12

20 09

15

2010

16

OV E R A L L H O S P ITA L

D I SC H A RG E

M O RTA L IT Y

R ATE 8

S T S N AT I O N A L

B E N C H M A R K 9 4.1%

0%

Norwood operationVolume by year

2014

17

2013

17

2012

21

20 06

18

20 07

18

20 08

28

20 09

15

2010

17

2011

24

OV E R A L L H O S P ITA L

D I SC H A RG E

M O RTA L IT Y

R ATE 8

S T S N AT I O N A L

B E N C H M A R K 9 15.7%

12.3%The Congenital Heart Surgery Service performs an average

of 20 Norwood operations per year for hypoplastic left

heart syndrome and other single ventricle anomalies.

The arterial switch operation (ASO) is offered to patients

with simple transposition of the great arteries, complex

transposition of the great arteries and Taussig-Bing

anomaly. Historically, this operation has not been viewed as

a viable treatment option for some children due to their

size, complex coronary anatomy and associated cardiac

malformations. Our outcomes have demonstrated that

these characteristics are not associated with increased

operative risk. Our hospital survival rate is 100 percent.

8 Hospital mortality is calculated over the last five years from 2010-2014.9 STS benchmark data is from 2010-2013.

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H E A R T C E N T E R 2 0 1 4 15

The gift that keeps givingKathy and Anthony tried for years to have a baby. So when an ultrasound revealed that the daughter

Kathy was carrying had a dangerous heart defect, they immediately kicked into crisis mode. The

condition, dextro transposition of the great arteries, or dTGA, meant the two main arteries leading out

of the baby’s heart were switched or transposed. Surgery would be needed soon after birth.

After extensive homework, the couple booked several appointments with top pediatric

cardiovascular surgeons close to their home in Cleveland, Ohio. Then a physician told them about

Texas Children’s, and they found themselves traveling more than a thousand miles to take a look.

“Dr. Fraser was warm, knowledgeable and took the time to answer all our questions,” Kathy says.

“It was a difficult decision to be so far from home, and Texas was the least convenient option,”

Anthony says. “But the results Dr. Fraser has had with dTGA and the confidence we have in him

speak for themselves.”

The couple moved into an apartment in Houston a couple of weeks before Olive was born

at Texas Children’s Pavilion for Women. Immediately after birth, she was whisked away to the

CVICU. At two days old, she had a catheter procedure called a septostomy. When Olive was eight

days old, Dr. Fraser performed a complex 13-hour surgery called an arterial switch to restore

regular blood flow through the heart and to the rest of the body.

“The next two weeks were really tough,” Anthony says. “It’s scary enough to be a new parent, and

it was hard to see her on a breathing machine and connected to so many monitors. But it was

extraordinary how much attention she got. Dr. Fraser came in every day, even on Sunday.”

Three weeks later, Olive was healthy enough to go home. Today she is an active 2-year-old, and

she has achieved every developmental and physical milestone expected for her age. She sees a

cardiologist in Cleveland, and Dr. Fraser reviews her scans. Olive will have to have another surgery,

and when she does, she will be back at Texas Children’s.

“She is our miracle,” Kathy says. “She has taught us to love every moment. Not a day goes by that

I don’t thank Dr. Fraser. You would think gratitude would fade, but it just grows.”

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H E A R T C E N T E R 2 0 1 416

Single Ventricle ProgramThe Single Ventricle Program (SVP) at Texas Children’s Hospital is a multidisciplinary team that

includes pediatric cardiologists, nurse practitioners, neurodevelopmental specialists, dietitians

and social workers. Patients have a variety of diagnoses such as hypoplastic left heart syndrome

(HLHS) or tricuspid atresia, but no matter the diagnosis, the program offers specialized medical

care, education and support. The SVP team strives to ensure the best possible outcomes for this

specialized patient population.

Many of the patients who enter the program are identified before delivery during their fetal

ultrasound and are seen at Texas Children’s Fetal Center™ at Texas Children’s Pavilion for Women.

We know that having a prenatal diagnosis is important for the family and for the infant during the

critical newborn period.10

One unique prenatal offering, “Parenting Your High-Risk Baby,” focuses on preparing for delivery

and parenting skills needed while in the hospital. The class is presented by multidisciplinary teams

from both the Pavilion for Women and Texas Children’s Heart Center. In the final session, parents

meet with other families who have already gone through the same experience.

After delivery, the newborn is transferred to the Cardiovascular Intensive Care Unit for further

care. The SVP team works collaboratively with other teams at Texas Children’s Hospital during the

transition from newborn care to open heart surgery to recovery. Many of the infants will be able

to go home, but some patients, identified for a variety of reasons, will need to stay in the hospital

until after their second surgery, which is usually a Bi-Directional Glenn.

As the infant recovers and progresses, the SVP team members educate and prepare the family

for the transition home. Since these infants are at risk for a number of medical complications,

parents are critical members of the team. Before discharge, parents receive education on the

Single Ventricle Home Monitoring Program. Parents learn how to keep track of their baby’s daily

progress by measuring daily weights, oxygen saturations, feeding information and heart rate.

Once parents learn and become comfortable

with daily care, the baby is discharged from the

hospital. We support them at home through

phone consultations and frequent outpatient clinic

follow-up visits. The SVP monitors for symptoms

referred to as “red flags.” When these symptoms

are noted, the patient is brought in for evaluation

and sometimes readmitted to the hospital. The

family has 24-hour access to a member of the team

for questions or concerns.

The SVP is also an active member of the National

Pediatric Cardiology Quality Improvement

Collaborative, a multi-hospital collaborative focused

on improving quality and outcomes for children with HLHS.

10 Morris SA, Ethen MK, Penny DJ, Canfield MA, Minard CG, Fixler DE, Nembhard WN. Prenatal diagnosis, birth location, surgical center, and neonatal mortality in infants with hypoplastic left heart syndrome. Circulation. 2013; CIRCULATIONAHA.113.003711. Published online before print October 17 2013.

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H E A R T C E N T E R 2 0 1 4 17

Outcomes of patients with Anomalous Aortic Originof a Coronary Artery (AAOCA)

Total patients

Surgery treatment

Mortality

Complications

90

29

0

2

Coronary Anomalies ProgramIn December 2014, Texas Children’s Hospital hosted its second national conference on coronary

artery anomalies. A group of rare congenital heart defects, coronary artery anomalies are

associated with coronary ischemia, myocardial infarction and sudden death. The condition is the

second-leading cause of sudden cardiac death in children and young adults.

Diagnosing these anomalies can be challenging because many individuals with the condition have

no symptoms. Those who do have symptoms complain most often of chest pain, palpitations,

dizziness or fainting during or just after exercise.

How to best treat a child or young adult with coronary artery anomalies is a subject of debate

in the medical community. Most physicians agree that surgery is necessary for patients who show

evidence of decreased blood flow to the heart tissue, but how to treat patients who have no

physical complaints – and who show no evidence of reduced blood flow to the heart – is unclear.

During the conference, speakers from 16 leading heart institutions provided a dedicated forum

to discuss the diagnosis and management of patients with coronary artery anomalies. A panel

discussion with families affected

by the condition brought special

attention to the psycho-social needs

of patients with cardiac anomalies

as well as their parents and siblings.

Other talks focused on the most

appropriate imaging modalities,

identification of risk factors, different

management strategies based on best

available evidence, surgical techniques,

and counseling of patients and families

regarding treatment and exercise

recommendations.

11Data as of January 31, 2014.

The program began tracking outcomes in December 2012 and as of February 2015 has recorded:

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H E A R T C E N T E R 2 0 1 418

11 VADs placed from 1995-2014.

Heart Failure and Transplant Program Since its inception in 1984, Texas Children’s Heart Center has performed more than 315 heart

transplants, making it one of the largest and most successful programs in the nation. As the

number of diagnoses for pediatric heart failure increases, so has our depth and breadth of

experience in treating patients, as has our ability to offer the right mechanical circulatory device

to support each child in heart failure. For more information on the Heart Transplant Program,

please visit texaschildrens.org/transplant.

Since 2011, Texas Children’s Heart Center has been an active member of the Pediatric Heart

Transplant Study, which is dedicated to the advancement of the science and treatment of children

during listing for and following heart transplantation. The purpose of the group is to establish

and maintain an international, prospective, event-driven database for heart transplantation, to use

the database to encourage and stimulate basic and clinical research in the field of pediatric heart

transplantation, and to promote new therapeutic strategies.

138TOTAL CASES

VAD implantations11 by device

23 22

44

17

11

3

11

3

Berlin Heart EXCOR® Pediatric

Thoratec P-VAD®

3Impella®

Rotaflow® Bio-Medicus®

TandemHeart®

1SynCardia®

Total Artificial Heart

HeartWare®

Micromed®

HeartMate II®

Mechanical circulatory support is often used as a bridge to transplantation in

children. At Texas Children’s Heart Center, we select a ventricular assist device

(VAD) from a variety of options to best meet the needs of the individual patient.

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H E A R T C E N T E R 2 0 1 4 19

Extracorporeal life support Extracorporeal life support (ECLS) is a treatment that helps support children when their heart

and/or lungs are unable to supply enough blood flow or oxygen to the body. This support may be

necessary after heart surgery or in some children with heart or lung disease. ECLS is provided

in the Pediatric Intensive Care Unit (PICU), Cardiovascular Intensive Care Unit (CVICU) or

Neonatal Intensive Care Unit (NICU) based on each child’s underlying problem. Our specially

trained team of cardiac intensivists, surgeons, critical care nurses and ECLS specialists carefully

monitors each child’s condition while on ECLS.

To provide ECLS, we use extracorporeal membrane oxygenation, or ECMO. With ECMO, blood

is pumped through the cannulae and into the machine oxygenating the patient’s blood. The

oxygenated blood is then returned through the circuit to the body, creating a continuous flow

and doing the work of the heart and/or lungs so the patient’s body can heal.

CVICU ECMO cases by year

2014

28

2013

22

2012

15

2011

18

2010

14

2009

8

2008

8

2007

14

The ECMO program at Texas Children’s Hospital received the Award for

Excellence in Life Support from the Extracorporeal Life Support Organization

and is a designated Center of Excellence.

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Berlin Heart EXCOR® Pediatric Ventricular Assist DeviceIn 2012, the United States Food and Drug Administration (FDA) granted humanitarian device

exemption for the Berlin Heart EXCOR® Pediatric Ventricular Assist Device, the only pediatric

mechanical circulatory support device designed specifically for infants and small children. This was

a landmark day for physicians who treat children dying of heart failure whose only hope is a heart

transplant. Small children awaiting heart transplantation face a long time on the waiting list due to

limited availability of donor organs. Mortality rate while waiting is approximately 25 percent due

to progressive system organ failure.

FDA approval began with a multi-year, multi-institution study, led in part by Texas Children’s

Hospital. In 2005, a trans-Atlantic dialogue was initiated among the Berlin Heart Corporation,

clinicians in North America and the FDA to design and conduct the first prospective pediatric

ventricular assist trial in the world. The study design addressed questions of safety and benefit

of the Berlin Heart in bridging children with heart failure until they received a heart transplant.

This study involved detailed analysis and ongoing multicenter data collection and coordination in

critically ill children with rapidly progressive heart disease.

During the course of the study, Texas Children’s implanted 27 Berlin Hearts – more than any

other center in the U.S. – and our results were encouraging. Findings from this study were

published in the New England Journal of Medicine12 in August 2012. Outcomes for 48 children

(infants through 16 years) who received the Berlin Heart from 2007-2010 were compared to

those of patients supported by extracorporeal membrane oxygenation.

12 Data includes patients who received heart-and-lung transplants simultaneously.

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H E A R T C E N T E R 2 0 1 4 21

Heart transplants13

Volume by year

2014

32

2013

15

2012

14

201115

2010

13

2009

18

2008

16

2007

11

Heart transplant patient survival rates14 Pediatric age <18

3 YEARS AFTER TRANSPLANT

1/1/ 09 TO 6 /30 /11 N =31

1 YEAR AFTER TRANSPLANT

7/1/11 TO 12 /31/13 N =31

TEXAS CHILDREN’S HEART CENTER SRTR EXPECTED NATIONAL

91.5

9%

90

.32

%

85

.87

%

94

.37

%

92

.47

%

92

.38

%

13 Data includes patients who received heart-and-lung transplants simultaneously.14 Scientific Registry of Transplant Recipients (SRTR). Program Specific Reports. Table 11 – srtr.org.

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0%

20%

40%

60%

80%

100%

ERA I

ERA III

ERA II

Years post transplant

Surv

ival

(%

)

5 10 15 20 25 300

76

59

151

46

38

49

34

27

0

27

3

15

0

2

ERA I: 1984-1998

ERA III: 2005-2014

ERA II: 1999-2004

15 Wilcoxen test 1984-1998 vs. 1999-2004: p < 0.9975, 1984-1998 vs 2005-2014: p < 0.0022, 1994-2004 vs. 2005-2014: p < 0.0026. Texas Children’s data: 1984-2014, 275 Txs on 286 patients, Age between 0 and 22 years. UNOS data: 1987-2013, 7437 Txs on 6487 patients (3 observations missing data), Age between 0 and 17 years.

Heart transplant survival curves The following graphs show the survival rates for patients by the number of years after heart

transplantation. The lines show the expected survival rates for our patients a number of years

after their transplant. Because these are estimates, the shaded areas show the 95 percent

confidence limits of the expected survival rates.

Survival curves15

by era

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H E A R T C E N T E R 2 0 1 4 23

0%

20%

40%

60%

80%

100%

1512,909

901,690

641,011

38475

1475

00

0 2 4 6 8 10

Years post transplant

Surv

ival

(%

)

UNOS

Texas

Children’s

Texas

Children’s

UNOS

Survival curve vs. UNOS16

Era III (2005-2014)

16 Wilcoxen test 1984-1998 vs. 1999-2004: p < 0.9975, 1984-1998 vs 2005-2014: p < 0.0022, 1994-2004 vs. 2005-2014: p < 0.0026. Texas Children’s data: 1984-2014, 275 Txs on 286 patients, Age between 0 and 22 years. UNOS data: 1987-2013, 7437 Txs on 6487 patients (3 observations missing data), Age between 0 and 17 years.

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Interventional Cardiology Increasing numbers of heart conditions can be treated with minimally-invasive catheter-based

interventions. Texas Children’s Heart Center, the largest program in the region to offer

interventional cardiac catheterization for infants, children and adolescents, performed 1,150

cardiac catheterization procedures in 2014. The full-service cardiac catheterization laboratory

performs every possible intervention, including:

• Balloon valvuloplasty

• Balloon angioplasty (using standard as well as “cutting” balloons)

• Stent placement (e.g. for pulmonary artery stenosis, coarctation of the aorta, etc.)

• Device closure of atrial septal defects, arterial and venous collaterals, coronary fistulae, etc.

• Device closure of patent ductus arteriosus, including in low birth weight and premature babies

• Transcatheter pulmonary valve replacement using the Melody® valve

In addition to these relatively common procedures, the interventional cardiology team at Texas

Children’s Hospital has developed cutting-edge treatment strategies for some rare and debilitating

conditions such as:

• Pulmonary vein stenosis

• Venous thrombosis

• Multiple muscular ventricular septal defects (“Swiss cheese” septum)

• Congenital portosystemic shunts (Abernethy malformation)

• Pulmonary arteriovenous malformations (congenital and acquired)

• Pulmonary hypertension

• Renal artery stenosis

• Mid-aortic syndrome

• Percutaneous ventricular assist device placement for heart failure (Impella® device)

• Fetal cardiac interventions for conditions such as critical aortic stenosis and hypoplastic left

heart syndrome

Despite being a regional and national referral center for the most complex forms of acquired

and congenital heart disease, our team has an extraordinarily low complication rate from cardiac

catheterizations (see chart on following page). We have a strong culture of attention to radiation

safety and continuous quality improvement, and we strive to promote these concepts locally and

nationally. Dr. Henri Justino, medical director of the Charles E. Mullins Cardiac Catheterization

Laboratories, serves as the national chair for the Pediatric Quality Improvement Committee of

the Society for Cardiovascular Angiography and Interventions. Our interventional team is actively

involved in research into new treatment strategies for congenital heart disease, and is invited to

lecture on these topics nationally and internationally.

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H E A R T C E N T E R 2 0 1 4 25

Cardiac catheterization laboratory complicationsby rate

Cases without complications

0.68%

1.10%

Cases withmajor complications

Cases withminor complications

98.22%

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Pioneering procedure gives baby healthy startWhen Kaylin made medical history, she weighed just over two pounds and had a heart the

size of a nickel. Born at 28 weeks gestation, Kaylin had critical aortic valve stenosis and critical

coarctation of the aorta, two areas of narrowing on the left side of the heart which forced it to

work too hard to deliver blood throughout her body. Without intervention, she would die – but

the procedure she needed had never been performed on a baby so small.

Texas Children’s Hospital, only 200 miles from the family’s home in Austin, had the expertise to

try the intervention. It was their best chance. Within a day, Texas Children’s Kangaroo Crew flew

mother and baby to Houston. The next day, physicians gathered to discuss the options.

“It felt like all the best minds were on the scene,” says Kaylin’s mother, Nancy. “I instantly knew

we were in the right place and that we had found a hospital that could help her.”

But she and her husband, Chris, were nervous nonetheless. After almost six hours, Nancy and

Chris got the good news that the procedure was a success. Interventional cardiologist Dr. Henri

Justino made an incision about the size of a pinprick to perform a balloon aortic valvuloplasty and

snaked a catheter through the carotid artery to repair the aortic valve and the narrowed aorta

with a tiny balloon and stent.

Kaylin spent a few days in the CVICU and then was flown back to Austin, where she stayed several

weeks in the NICU. Due to her rapid growth, she required two more catheterization procedures

through the femoral artery to redilate the aortic valve and the stent in her first year of life.

Another procedure at 2½ years was performed to intentionally fracture the stent, which Kaylin

had outgrown.

Now 8, Kaylin visits Texas Children’s annually. She will require additional procedures someday, but

for now, Kaylin is an active child who has never had any restrictions. She plays competitive soccer

and is above her grade level in math and reading.

“It’s amazing,” Nancy says. “You would never be able to tell by looking at her that she had such

a revolutionary minimally-invasive cardiac catheterization procedure as a baby. We owe so much

to Texas Children’s.”

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H E A R T C E N T E R 2 0 1 4 27

ElectrophysiologyThe Arrhythmia and Pacing Service at Texas Children’s Heart Center offers the full complement of

diagnostic and therapeutic options to evaluate and manage any heart rhythm abnormality. We have

one of the highest volume pediatric centers in the country for invasive electrophysiology studies

and pacemaker/defibrillator implantations, and we maintain success rates for ablations that exceed

the national average. Innovation, research and experience have made Texas Children’s Heart

Center one of the leading centers in the world for the diagnosis and treatment of arrhythmias in

children and adults with congenital heart disease.

Texas Children’s Hospital

98.3%

National benchmark17

95.2%

2 0 1 4

AC U T E S U C C E S S R AT E

Supraventricular tachycardia ablation outcomes

17 Based on data from the Pediatric Radiofrequency Catheter Ablation Registry. Kugler JD, Danford DA, Houston KA, et al. Pediatric radiofrequency catheter ablation registry success, fluoroscopy time, and complication rate for supraven-tricular tachycardia: comparison of early and recent eras. J Cardiovasc Electrophysiol. 2002 Apr; 13(4):336-41.

18 Kim JJ, Macicek SL, Decker JA, Kertesz NJ, Friedman RA, Cannon BC. Magnetic versus manual catheter navigation for ablation of free wall accessory pathways in children. Circulation: Arrhythmia and Electrophysiology. 2012 Aug; 5(4):804-8.

Stereotaxis Texas Children’s Hospital is the only freestanding children’s hospital in the U.S. with a remote

magnetic navigation (MN) system called stereotaxis for catheter manipulation. Stereotaxis has

been utilized for 63 ablation cases over the last three years.

MN offers theoretic advantages in the treatment of children. The catheter is more flexible and

atraumatic than standard catheters, potentially eliminating risk of heart perforation. It can also be

navigated with more precision, allowing for movements as small as one millimeter or deflections

as small as one degree. This may result in enhanced accuracy and safety during mapping and

ablation of certain arrhythmias in this patient population.

We performed a review of data in children undergoing radiofrequency ablation at our institution.18

The efficacy and safety of an MN-guided approach and standard manual techniques for mapping

and ablation of AP-mediated tachycardia were compared. Acute success rates and recurrence

rates were equivalent (p=NS). There was also no difference in total procedure time. However,

fluoroscopy time was significantly reduced in the MN group when compared to the manual group

(p < 0.001). There were no complications in either group.

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Additionally, in order to decrease morbidity associated with radiation exposure during ablation

cases, the Arrhythmia and Pacing Service has established protocols utilizing non-fluoroscopic

mapping techniques to minimize radiation. This has resulted in a three-fold reduction in

fluoroscopy times with 97 procedures being performed with minimal radiation (<1 minute) over

the past two years.

2013

18.9 minutes

2014

4.2 minutesAV E R AG E F L U O RO T I M E

Average fluoro time for ablations

Changes in fluoroscopy time for ablation proceduresby year

0%

25%

50%

75%

100%

1/4/12

4/3/12

6/6/12

8/20

/12

11/7/12

12/19/12

2/27

/13

4/3/13

5/24

/13

7/3/13

8/9/13

11/4/13

12/30/13

2/7/14

3/18

/14

6/18

/14

7/30

/14

9/15

/14

11/21/14

1/1/15

2/18

/15

UCL

UCL

UCLUCL

Mean

Mean

Mean Mean

Date of procedure

Tim

e in

min

utes

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H E A R T C E N T E R 2 0 1 4 29

Diagnostic Testing and Cardiac Imaging Each year, our non-invasive imaging lab performs more than 24,000 echocardiograms for fetuses,

infants, children and adults with congenital or acquired heart disease. The imaging lab provides

transesophageal, epicardial, intracardiac and transthoracic echocardiogram support in the

catheterization labs and operating rooms on a daily basis. The main echocardiography lab consists

of 10 rooms with a four-bed sedation bay and a state-of-the-art physician work area that houses

five reading stations.

Fetal cardiac interventionsFor some fetuses with cardiovascular conditions, fetal cardiac intervention is available at Texas

Children’s Hospital to try to improve the physiology, even before birth. Texas Children’s Fetal

Center™ at Texas Children’s Pavilion for Women is one of only a few centers in the world capable

of providing the full array of fetal cardiac interventions, which involves a coordinated effort among

a large, multidisciplinary team of fetal cardiologists, obstetricians and gynecologists, interventional

cardiologists, congenital heart surgeons, fetal imaging experts, maternal and fetal anesthesiologists

and other clinical specialists. We offer the following fetal cardiac interventions:

• Fetal arrhythmia evaluation and treatment – While some abnormal fetal rhythm problems are

benign, some may result in fetal heart failure and can pose a risk to the fetus and mother. We

offer a variety of therapies that are customized for both the fetus and mother. We collaborate

closely with our adult cardiologists to ensure both fetus and mother are safe during treatment.

• Fetal hypoplastic left heart syndrome (HLHS) and related conditions – We offer three fetal

cardiac interventions where the left heart structures are small, and the type of therapy

depends on the fetal heart anatomy. For fetal aortic stenosis, which progresses to HLHS, we

offer aortic valvuloplasty. This procedure is performed by a multidisciplinary team and consists

Fetal echocardiogramsVolume by year

20142013201220112010200920072005

200

400

600

800

1000

1200

1400

1600

1800

2000

20082006

54

3

53

8

60

7 76

7 95

3

89

9

94

6

1,2

53

1,5

92 1,

781

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H E A R T C E N T E R 2 0 1 430

of placing a small balloon across the fetal aortic valve to enlarge it and to promote blood flow

through the left side of the heart.

• Fetal atrial septal intervention – We offer this procedure for fetuses with hypoplastic left heart

syndrome or mitral valve dysplasia and a restrictive or intact atrial septum. For this procedure,

either a balloon or stent is placed across the atrial septum of the heart to decrease the

pressure in the left atrium, and to help the fetal lungs recover before birth.

Some fetuses have small left-sided structures and without intervention will need neonatal heart

surgery, but do not meet the criteria for the above procedures. We are currently conducting an

experimental study protocol in which we provide daily home oxygen to mothers, also known as

maternal hyperoxygenation, to promote fetal left heart growth.

Stress MRI There is a growing need within pediatrics for the accurate assessment of myocardial perfusion

due to both acquired and congenital heart defects. At Texas Children’s Hospital, we are one of

the few pediatric centers in the country to offer stress MRI by multiple pharmacologic stress

agents. This technology has been shown to be superior to many other non-invasive modalities

in the adult population, and Texas Children’s Hospital is adopting many of these same principles

and applying them to the pediatric population. These examinations involve the dedicated and

coordinated efforts of a pediatric cardiologist, pediatric radiologist, cardiac pharmacologist, cardiac

MRI technician and MRI physicist.

We have performed nearly 25 of these examinations over the last academic year with excellent

results and no adverse effects. Some of the different pathology types include D-transposition of

the great arteries after arterial switch procedure, aortic valve replacement by the Ross procedure,

repaired anomalous origin of the coronary arteries, Kawasaki disease and myocardial bridge. The

possible pharmacologic agents include either regadenoson, adenosine or dobutmaine, and the

decision making is dependent upon the patient’s disease type and their size.

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Adult Congenital Heart Disease ProgramThe Adult Congenital Heart Disease (ACHD) Program offers a single point of care for patients

with congenital heart disease from birth to adulthood. Our program offers the full range

of ACHD services and specialties including surgery, diagnostic and interventional cardiac

catheterization, noninvasive imaging (including cardiac MRIs and coronary CT angiography),

arrhythmia services, and more.

ACHD clinic encountersin 2014

Clinic visits

Unique patients 1,186

1,606

ACHD program volumes19

by procedure

5% 9%

86%

C A R D I AC M R I = 1 1 7

E C H O C A R D I O G R A M S = 2 , 0 9 2

E P & I N T E RV E N T I O N A L P RO C E D U R E S = 2 1 5

19 Volumes reflect patients age 18 years or older.

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Cardiac Resource Utilization in Adults at a Freestanding Children’s Hospital20, 21

Despite the growing population of adults with congenital heart disease, a gap remains in published

information looking at the care these patients receive at pediatric institutions. With this in mind, a

group of cardiologists at Texas Children’s Hospital recently evaluated the experience and resource

utilization these adult patients had at our institution from January 2006 through December 2010.

20 Data collected from Jan. 2006 – Dec. 2010.21 Ermis P, DietzmanT, Franklin W, Kim J, Moodie D, Parekh D. Cardiac resource utilization in adults

at a freestanding children’s hospital. Congenit Heart Dis. 2014, 9(3): 178-186.

Adult patients (18 years or older) 2,794

Mean age 23.5 +/- 8.4 years

% with congenital heart disease 62.6%

Procedures Number Minor adverse events

Major adverse events

Mortality

Catheterization lab 525 4.8% 0.7% 0%

Surgical procedures 104 6.7% 1.9% 0%

Visits NumberClinic 5,489

Emergency department 162

Diagnoses (most common) Number PercentageTetralogy of Fallot 228 13%

D-transposition of the great arteries 208 11.9%

Single ventricle physiology 187 10.7%

Atrial septal defect 128 7.3%

Ventricular septal defect 117 6.7%

Simple

Severe Moderate

41%

37%22%

Complexity of congenital heart disease

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H E A R T C E N T E R 2 0 1 4 33

Cardiovascular Anesthesia Pediatric Cardiovascular Anesthesia at Texas Children’s Hospital provides sedation and anesthesia

services for all congenital cardiac operations and for children with congenital heart disease

undergoing various non-cardiac procedures. Services are also provided for catheterization and

electrophysiology procedures as well as imaging studies in MRI, CT and interventional radiology.

The Pediatric Cardiovascular Anesthesia Division treated more than 2,600

patients in 2014. Beyond anesthetic care, the division actively participates in

the daily care of patients in the Cardiovascular Intensive Care Unit.

Cardiovascular anesthesia casesby location

M R I

I N T E RV E N T I O N A L R A D I O L O G Y

C O N G E N I TA L H E A RT S U R G E RY O P E R AT I N G RO O M S

C AT H E T E R I Z AT I O N A N D E L E C T RO P H YS I O L O G Y

2014

1,09

41,

002

152

430

2010

99

1

82

6

10

9

36

2

2 012

92

4

83

9

10

9

34

7

2011

95

5

85

5

12

5

37

5

2 013

88

0

91

0

18

4

46

2

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2014Cardiovascular anesthesia use during MRI by age

18 yearsor older

6%

31 days-1 year

14%

1-6 years

44%

7-13 years

28%

0-30 days

8% 6%6%%%%

3

1414%

11-6

4

arss

%%

8%8%

Cardiovascular anesthesia duration during MRI

L E S S T H A N 1 H O U R

1 - 2 H O U R S

2 - 3 H O U R S

M O R E T H A N 3 H O U R S

74%

6%19%

1%

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H E A R T C E N T E R 2 0 1 4 35

Leaders in cardiovascular anesthesia education

Transesophageal Echocardiography for Congenital Heart Disease, edited by Texas

Children’s physicians Pierre C. Wong, M.D., and Wanda C. Miller-Hance, M.D.,

focuses on the application of imaging modality in patients with congenital

heart disease. Published in early 2014, this work provides practical and

instructive information, supplemented with figures/illustrations and

high-quality videos. It is a resource for clinicians and trainees who wish to

obtain basic knowledge or advance their understanding of the field.

Gregory’s Textbook of Pediatric Anesthesia introduces the basics of the field and

explains how they are applied to contemporary practice both in and out of

the operating room. Co-edited by Dean B. Andropoulos, M.D., chief of

Anesthesiology at Texas Children’s Hospital, the book uses an evidence-based

approach and is supplemented by in-depth case studies that spotlight

best practice in action across all the major subspecialties. It also contains

extensive information on anesthesia care for patients with congenital

heart disease.

Anesthesia for Congenital Heart Disease, edited by Dean B. Andropoulos, M.D.,

chief of Anesthesiology, as well as Texas Children’s Hospital anesthesiologists

Stephen A. Stayer, M.D., and Emad B. Mossad, M.D., is the leading textbook in

the field of pediatric cardiac anesthesia. This textbook contains information

on the rapid advances in surgery and anesthesia for congenital heart disease

that have occurred in recent years, including neonatal cardiac surgery and

anesthesia, treatment of single ventricle patients, new diagnostic modalities, and

developments in the catheterization laboratory. It also addresses mechanical

support of circulation, embryology and quality/outcomes management.

Following the inaugural American Board of Anesthesiology Pediatric Anesthesiology

subspecialty board exam, Texas Children’s Hospital is now home to more than 60

subspecialty board-certified pediatric anesthesiologists.

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22 Andropoulos DB, Hunter JV, Nelson DP, et al. Brain immaturity is associated with brain injury before and after neonatal cardiac surgery with high-flow bypass and cerebral oxygenation monitoring. J Thorac Cardiovasc Surg. 2010; 139(3):543-56.

23 Andropoulos DB, Ahmad HB, Haq T, Brady K, Stayer SA, Meador MR, Hunter JV, Rivera C, Voigt RG, Turcich M, He CQ, Shekerdemian LS, Dickerson HA, Fraser CD, Dean McKenzie E, Heinle JS, Blaine Easley R. The association between brain injury, perioperative anesthetic exposure, and 12-month neurodevelopmental outcomes after neonatal cardiac surgery: A retrospective cohort study. Paediatr Anaesth. 2014 Mar;24(3):266-74. doi: 10.1111/pan.12350. PubMed PMID: 24467569.

24 Rios DR, Welty SE, Gunn JK, Beca J, Minard CG, Goldsworthy M, Coleman L, Hunter JV, Andropoulos DB, Shekerdemian LS. Usefulness of routine head ultrasound scans before surgery for congenital heart disease. Pediatrics. 2013 Jun;131(6):e1765-70.

25 Beca J, Gunn JK, Coleman L, Hope A, Reed PW, Hunt RW, Finucane K, Brizard C, Dance B, Shekerdemian LS. New white matter brain injury after infant heart surgery is associated with diagnostic group and the use of circulatory arrest. Circulation. 2013 Mar 5;127(9):971-9.

26 Andropoulos DB, Easley RB, Brady K, McKenzie ED, Heinle JS, Dickerson HA, Shekerdemian L, Meador M, Eisenman C, Hunter JV, Turcich M, Voigt RG, Fraser CD Jr. Changing expectations for neurological outcomes after the neonatal arterial switch operation. Ann Thorac Surg. 2012 Oct;94(4):1250-5.

27 Gunn JK, Beca J, Hunt RW, Olischar M, Shekerdemian LS. Perioperative amplitude-integrated EEG and neurodevelopment in infants with congenital heart disease. Intensive Care Med. 2012 Sep;38(9):1539-47.

28 Beca J, Gunn J, Coleman L, Hope A, Whelan LC, Gentles T, Inder T, Hunt R, Shekerdemian L. Pre-operative brain injury in newborn infants with transposition of the great arteries occurs at rates similar to other complex congenital heart disease and is not related to balloon atrial septostomy. J Am Coll Cardiol. 2009 May 12;53(19):1807-11.

29 Andropoulos DB, Mizrahi EM, Hrachovy RA, et al. Electroencephalographic seizures after neonatal cardiac surgery with highflow cardiopulmonary bypass. Anesth Analg. 2010; Jun 110 (6): 1680-5.

30 Fraser CD 3rd, Brady KM, Rhee CJ, Easley RB, Kibler K, Smielewski P, Czosnyka M, Kaczka DW, Andropoulos DB, Rusin C. The frequency response of cerebral autoregulation. J Appl Physiol. 2013;115:52-6.

31 Pesek M, Kibler K, Easley RB, Mytar J, Rhee C, Andropoulos D, Brady K. The upper limit of cerebral blood flow auto-regulation is decreased with elevations in intracranial pressure. Neurosurgery. 2014 Apr 15. [Epub ahead of print]

Developmental Outcomes Texas Children’s Heart Center strives to offer the best neurodevelopmental outcomes for

our patients. As leaders in the field, we are focused on improving our patients’ long term

developmental outcomes through state-of-the-art monitoring, focused research and routine

neurodevelopmental follow-up of our highest risk patients.

Cerebral autoregulation monitoring During surgery, our pediatric cardiovascular anesthesiologists work closely with surgeons and

perfusionists to provide consistent care for cardiopulmonary bypass and anesthetic management.

In an effort to continue improvement of cardiac outcomes, research teams are investigating

a method to optimize cerebral oxygenation and brain blood flow through a new method of

monitoring called cerebral autoregulation.

This unique, non-invasive approach has been designed by our cardiovascular anesthesiologists to

monitor in real time the adequacy of blood flow to the brain during the highest risk parts of open

heart surgery. We aim to be able to individualize cardiac bypass to provide ideal conditions to

every patient at every moment during their heart operations. Our patented technology is being

tested with the ultimate goal of incorporation into pediatric operating rooms across the world.

Long-term neurodevelopmental outcomes Heart Center investigators have extensive experience in prospective outcomes research22-31

in infants undergoing heart surgery. We have published extensively on this topic in high-impact

cardiac journals, and have presented our findings at many national and international meetings.

Our teams have already uncovered important factors that contribute to developmental outcome,

including brain immaturity, early brain injury and anesthetic drug exposure. The long-term impact

of early brain injury will be better understood as we continue to follow patients throughout

childhood. This will also form the basis of research aimed at providing early interventions to

minimize the risk of brain injury and impaired development.

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Cardiac Developmental Outcomes ProgramUntil recently, most neurodevelopmental follow-up has been in the context of research studies.

Texas Children’s Heart Center’s Cardiac Developmental Outcomes Program focuses on medical,

social and developmental health in order to help children reach their maximum potential.

Launched in April 2013, the outcomes program was created to provide routine neurodevelopmental

assessments and referrals to babies and children with congenital heart disease who have

undergone early surgery. During its first year of operation, the team assessed more than 100

babies and children and provided referrals to a variety of specialists including speech and language,

hearing, neurology and behavioral experts. Previously, these early interventions would not have

been readily available.

The outcomes team includes developmental specialists and psychologists who work with the

Heart Center to identify any problems at the earliest possible stage. This provides an opportunity

for early intervention in order to minimize the impact of issues during childhood.

The program provides family-centered care, where the child’s parents or caregivers are directly

consulted and involved in their child’s assessment and interventions for developmental progress.

We also help families find resources that support their child’s development in their communities.

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Cardiovascular Intensive Care Unit The Cardiovascular Intensive Care Unit (CVICU) team of cardiac intensivists, nurses, respiratory

therapists, social workers, dietitians and support staff provide intensive care to newborns, infants,

children and young adults with heart disease. One of the largest of its kind in the country, the

21-bed CVICU cares for patients undergoing surgery for congenital heart disease, as well as those

with end-stage heart failure before and after heart transplantation. In 2014, our CVICU medical

team welcomed five new faculty members and continues to expand to support our growing

patient population and increasing patient complexity.

Education in the CVICUThe faculty of the CVICU represent a multidisciplinary team focused on the care of the most

critically ill patients with cardiac disease. Paul A. Checchia, M.D., serves as president of the

Pediatric Cardiac Intensive Care Society (PCICS), the largest international society of its kind.

Dr. Checchia and Ronald Bronicki, M.D., have led the international symposium for the PCICS for

the past eight years. Faculty also serve as editors of the leading textbooks and journals in the field,

including the journal Pediatric Critical Care Medicine and the Rogers’ Textbook of Pediatric Intensive Care, 5th Edition.

The CVICU prepares future leaders in the field through a subspecialty training program in

Cardiovascular Critical Care Medicine. With four positions open every year, this is the largest

program of its kind in the world. Texas Children’s Hospital also developed the first subspecialty

training program in Neonatal Cardiovascular Medicine.

Nursing care in the Cardiovascular Intensive Care UnitThe CVICU at Texas Children’s Hospital has 106 specialized pediatric intensive care registered

nurses on staff. The hours per patient day equivalent in the CVICU are 22.43 compared to the

national benchmark of 22.1. Benchmark data is pulled from Children’s Hospital Association of like

units. All newly admitted surgical cases receive a minimum ratio of 1:1 nursing care. Ninety-two

percent of the CVICU registered nurses have a bachelor’s degree in nursing with a 35 percent

rate of attainment of Certification in Critical Care Nursing.

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Cardiovascular Intensive Care Unit admissions by age

19 years

5%

1 month-2 years

41%

2-6 years

14%

6-13 years

15%

13-19 years

11%

0-1 month

14%

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Quality, Innovation and ResearchTexas Children’s Heart Center is committed to examining the quality of care we provide to

patients and helping them understand our performance compared to national standards. We are

active participants in the following national quality programs:

• American College of Cardiology IMPACT (Improving Pediatric and Adult Congenital

Treatment) Registry™ for diagnostic and interventional catheterization procedures

• National Cardiovascular Data Registry’s ICD Registry measuring and improving care for

patients receiving implantable cardioverter defibrillators (ICDs)

• Society of Thoracic Surgeon’s National Quality and Outcomes program for congenital

heart surgery

Outcomes and Impact ServiceThe focus of the work in the Outcomes and Impact Service at Texas Children’s Hospital is to

measure clinical outcomes that matter to patients and the impact of care on the quality of life

and functional status of our patients over time. Our ultimate goal is to deliver the highest value of

health care to the patient.

By tracking outcomes, we learn about what happens to our patients, and we also learn about our

performance as a health care delivery organization. We know we must continue to strive toward

excellence in care delivery. In addition to providing statistics, we strive to engage patients and

families in a conversation about the choices they face that will impact their health.

Our team includes outcomes nurses dedicated to different clinical specialties, computer

programmers, data architects and specialists, and a statistician who works closely with clinical

and administrative teams to measure, improve and share our outcomes. The goals of our service

are to:

• Track and improve our clinical outcomes

• Understand the impact of those outcomes on the lives of our patients and families over time

• Make our outcomes data available in a form accessible by the general public

• Partner with patients and families in understanding outcomes data

• Help patients and families know what questions to ask when seeking medical treatment

Congenital Cardiac Anesthesia Society DatabaseFormed in January 2010 in coordination with the Society of Thoracic Surgeons Congenital Heart

Database, the Congenital Cardiac Anesthesia Society (CCAS) Database captures and reports data

concerning anesthesia monitoring and management, as well as adverse outcomes for congenital

heart disease patients. This population is significantly more likely to suffer adverse events and

cardiac arrest than patients without congenital heart disease.

Texas Children’s Hospital and other participating institutions use the data collected to determine

patterns and to track adverse outcomes relative to the national experience. Local data has

been used to determine the frequency of need for reintubation following in-OR extubation

after congenital heart surgery and to identify unfavorable outcomes associated with the use of

transesophageal echocardiography.

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Cardiovascular Simulation Program This in situ simulation program was developed to ensure our staff is prepared for patient

emergencies and other high-risk clinical scenarios. The goals of the program are to improve

patient safety through early identification of patient issues and to improve crisis resource

management and teamwork skills. Program leaders established a curriculum and learning matrix

in order to track objectives and progress. In addition to the “just-in-time” simulations done in

the new, in situ simulation room on the cardiology ward, ECMO simulations are scheduled in the

hospital’s state-of-the-art Simulation Center. In these scenarios, a large multidisciplinary team

consisting of a surgeon, perfusionist and medical team with nurses and physicians gathers for four

hours to simulate these time-sensitive, highly critical and technical events.

Pediatric Heart NetworkThe Pediatric Heart Network (PHN) was established in 2001 by the National Heart, Lung and

Blood Institute to improve health outcomes in patients with pediatric acquired and congenital

heart disease; to disseminate collaborative findings as the basis for improved evidence-based

treatment options and standards of care; to train and educate new investigators; and to provide

support and advocacy for families during the conduct of excellent, ethical clinical research.

The network of nine clinical sites is comprised of experienced research teams at hospitals that

specialize in the care of pediatric patients with heart disease and have a data coordinating center.

Texas Children’s Hospital and Baylor College of Medicine became a core site in September 2011.

Texas Children’s is committed to participation in all PHN studies, including five current and six

approved future studies. Current studies include the following:

• A study comparing two drug therapies (Beta Blocker Therapy (Atenolol) versus Angiotensin II

Receptor Blocker Therapy (Losartan)) on Marfan syndrome patients to see which is better at

slowing the speed of aortic enlargement. It will also compare side effects. The study began in

February 2007 and will take approximately seven years.

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• A study on Marfan syndrome patients to see if circulating TGF Beta levels correlate with

treatment arm (atenolol versus losartan) and/or clinical outcomes in the PHN Trial of Beta

Blocker Therapy (Atenolol) versus Angiotensin II Receptor Blocker Therapy (Losartan). By

measuring the circulating TGF Beta levels after achieving steady-state optimal dosing in this

trial cohort, we will be able to determine if there is a differential effect on TGF Beta levels in

the two treatment arms, and if circulating TGF Beta levels correlate with clinical response in

tissues where TGF Beta has a demonstrated (aorta, mitral valve) or proposed (left ventricle,

skeleton) contribution to disease pathogenesis.

• An ECHO Z-score study will develop normal reference values for echocardiogram

measurements obtained from multiple centers and geographic locations and adjusted for body

size, age, gender and race that currently do not exist.

• The inter-stage monitoring project will develop predictive models of impending

cardiorespiratory deterioration that will reduce morbidity and mortality in children with

hypoplastic left heart syndrome and related cardiac lesions. Using sophisticated computational

algorithms, we will develop multivariable predictive risk models that will analyze the

physiological data that are routinely available in our patients in real time, providing clinical staff

with early warning that a life-threatening event is imminent. This predictive risk model will

ultimately be applicable to other patient populations, and across PHN institutions and in other

cardiac centers caring for infants with critical congenital heart disease.

• Residual Lesion Score (RLS Main Study): a multi-center prospective study will validate the

RLS as a tool to predict early and mid-term outcomes for five common congenital cardiac

operations. The RLS study will develop the first validated tool for measurement of residual

lesions following congenital heart surgery. The proposed project will also lay the groundwork

for future projects, facilitating continuous quality improvement in congenital heart surgery.

Approximately 250 subjects per procedural category will be enrolled at each institution over a

two-year period. The study will last five years.

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Preventive Cardiology ProgramFocused on medically managing the risk factors of acquired heart disease in pediatric patients,

the Preventive Cardiology Program cares for patients who have abnormal lipid levels or blood

pressure results or who have been diagnosed with Kawasaki disease. The clinic’s goals are to

prevent future cardiac disease and events such as heart attack and stroke through early and timely

interventions and to learn how to better care for children with these issues.

ResearchTexas Children’s Heart Center is expanding the boundaries of pediatric cardiac care through

novel research. To learn more about how we are translating research into clinically relevant and

beneficial solutions for our patients, visit texaschildrens.org/heart.

Real-time predictive monitoring in ICU settingsThis research involves creating computer programs that look for early warning signs of

deterioration in intensive care unit patients. The programs process the physiological data recorded

from patient monitoring equipment in real time. The goal is to create a system that provides

advanced warning that a life-threatening event is likely to occur within the next 12-24 hours. Care

providers can use this information to proactively decide how to intervene before a situation

becomes life threatening, thus improving patient outcomes.

Genomic analysis of cardiac ICU patientsThis research aims to develop predictive outcome models in the unique patient population

of children with complex cardiac disease using novel genomic technology. The goal is to tailor

medical and surgical therapies, developmental therapy services, and even sedation strategies for

each individual child based on their own genetic profile.

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Charles D. Fraser, Jr., M.D., is surgeon-in-chief, co-director of Texas

Children’s Heart Center and chief of the

Division of Congenital Heart Surgery at

Texas Children’s Hospital. His academic

appointments include professor of Surgery

in the Michael E. DeBakey Department

of Surgery (tenured) at Baylor College of

Medicine, professor of Pediatrics at Baylor

College of Medicine and adjunct professor of

Bioengineering at Rice University. Dr. Fraser

holds the Clayton Chair in Surgery and the

Donovan Chair in Congenital Heart Surgery

at Texas Children’s Hospital. Dr. Fraser has

a clinical appointment at the Texas Heart

Institute, where he serves as director of the

Adult Congenital Heart Surgery Program.

Dr. Fraser’s extensive education began as an

undergraduate at The University of Texas at

Austin, where he graduated with honors in

mathematics. He received his medical degree

with honors from The University of Texas

Medical Branch at Galveston. His residency

and fellowship training took place at The John

Hopkins Hospital in Maryland. He completed

additional fellowship training in congenital

heart surgery at the Royal Children’s Hospital

in Melbourne, Australia. After joining the

faculty at the Cleveland Clinic, Dr. Fraser was

recruited to Texas Children’s Hospital in July

of 1995 to establish a dedicated pediatric

congenital heart surgery program.

Daniel J. Penny, M.D., Ph.D., M.H.A., is chief of Cardiology at Texas Children’s

Hospital, co-director of Texas Children’s Heart

Center and professor of Pediatrics at Baylor

College of Medicine. Dr. Penny obtained his

medical degree from the National University

of Ireland and received a doctoral degree

for his thesis “Functional Analysis of Fontan

Circulation” from the University of London.

He completed fellowship training in pediatric

cardiology at the Royal Children’s Hospital in

Melbourne and received a second doctoral

degree at Monash University, Australia for

his studies on neonatal circulation. Dr. Penny

worked as a pediatric cardiologist at the Royal

Brompton Hospital in London and at Great

Ormond Street Hospital in London. Before

coming to Texas Children’s Hospital, he was

the chief of Cardiology at the Royal Children’s

Hospital of Melbourne and a professor in the

Department of Pediatrics within the University

of Melbourne.

Meet Our Leaders

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Emad B. Mossad, M.D., is director of

Pediatric Cardiovascular Anesthesiology at

Texas Children’s Hospital and co-director of

Texas Children’s Heart Center. He is also a

professor of Anesthesiology and Pediatrics

at Baylor College of Medicine. Dr. Mossad’s

training began as an undergraduate at Cairo

University, Egypt. His medical degree is

from University of Cairo Medical School,

and his residency and fellowship training

in cardiac anesthesia took place at the

Cleveland Clinic Foundation. Dr. Mossad also

completed a pediatric anesthesia fellowship

at Children’s National Medical Center of

George Washington University and an

echocardiography fellowship at the Cleveland

Clinic. Dr. Mossad was the director of

Congenital Cardiac Anesthesia at Cleveland

Clinic for 13 years before he joined the

Cardiovascular Anesthesiology team at Texas

Children’s Hospital in 2008. Dr. Mossad is

a founding board member and currently

serves as President of the Congenital Cardiac

Anesthesia Society.

Lara S. Shekerdemian, M.D., F.R.A.C.P., M.H.A., is chief of Critical

Care and co-director of Texas Children’s

Heart Center. She also serves as the vice

chair of Clinical Affairs for the Department

of Pediatrics and professor of Pediatrics at

Baylor College of Medicine. She graduated

from Birmingham University Medical

School in 1990. She was awarded her

postgraduate doctoral degree for her

thesis, “Cardiopulmonary Interactions

in Congenital Heart Disease” from the

University of Birmingham in 1997.

Dr. Shekerdemian trained in pediatrics

and pediatric cardiology and undertook

postgraduate research in London. She

trained in critical care in London and

Toronto. She was previously on faculty at

Great Ormond Street Hospital in London

and was most recently the chief of Critical

Care at The Royal Children’s Hospital

in Melbourne. Her research and clinical

interests include extracorporeal life support,

brain injury in infants and children with

heart disease, and outcomes in children

after admission to the intensive care unit.

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Texas Children’s Heart Center Medical StaffTo view online profiles for each of our physicians, visit texaschildrens.org/heart.

Congenital Heart SurgeryCharles D. Fraser, Jr., M.D., ChiefIki Adachi, M.D.

Jeffrey S. Heinle, M.D.

Lauren Kane, M.D.

E. Dean McKenzie, M.D.

Carlos Mery, M.D.

Pediatric CardiologyDaniel J. Penny, M.D., Ph.D., M.H.A., ChiefHugh Allen, M.D., Ph.D.

Carolyn A. Altman, M.D., F.A.C.C., F.A.S.E.

Rachel Arnold, P.A.-C.

Nancy A. Ayres, M.D., F.A.C.C., F.A.S.E.

Manish Bansal, M.D.

Judith A. Becker, M.D.

Tasha Bryant, R.N., F.N.P.-C.

Antonio G. Cabrera, M.D.

Leigh Carreon, R.N., P.N.P.-A.C.

Constance Cephus, M.S.N., C.P.N.P.

Lisa D’Alessandro, M.D.

Caridad De La Uz, M.D.

Susan W. Denfield, M.D.

Heather A. Dickerson, M.D.

William J. Dreyer, M.D., F.A.C.C., F.A.A.P.

Peter Ermis, M.D.

Yuxin Fan, M.D.

Wayne J. Franklin, M.D.

Angela Gooden, R.N., P.N.P.-A.C./P.C.

Mary Hoang, R.N., P.N.P.-A.C./P.C.

Ben Jacob, P.A.-C.

Aamir Jeewa, M.D.

Henri Justino, M.D., C.M., F.R.C.P.C.,

F.A.C.C., F.S.C.A.I.

Josh A. Kailin, M.D.

Asra Khan, M.D.

Jeffrey J. Kim, M.D.

Michele Krenek, R.N., F.N.P.-C.

Julie K. Kuzin, R.N., P.N.P.-A.C./P.C.

William Buck Kyle, M.D.

Wilson Lam, M.D.

Renna Lange, R.N., C.P.N.P.

M. Regina Lantin-Hermoso, M.D.

Emily J. Lawrence, D.O.

Katherine Lindle, M.D.

Aimee Liou, M.D.

Keila N. Lopez, M.D., M.P.H.

Allison Mahlstedt, P.A.-C.

Shiraz A. Maskatia, M.D.

Wanda C. Miller-Hance, M.D.

Christina Miyake, M.D.

Silvana M. Molossi, M.D., Ph.D.

Douglas S. Moodie, M.D., M.S.

Shaine A. Morris, M.D., M.P.H.

Taylor Morse, R.N., P.N.P.-A.C.

Antonio R. Mott, M.D.

Michael R. Nihill, M.D.

Cory V. Noel, M.D.

Richard E. O’Brien, P.A.-C.

Elena C. Ocampo, M.D., F.A.A.P., F.A.C.C.

Dhaval R. Parekh, M.D.

Ricardo H. Pignatelli, M.D.

Jack Price, M.D.

Athar M. Qureshi, M.D.

Surabhi Chandra-Bose Reddy, M.D.

Priscila P. Reid, R.N., F.N.P.-C., P.N.P.-A.C.

Alan F. Riley, M.D.

Shannon M. Rivenes, M.D.

Alexia B. Santos, M.D.

David W. Sapire, M.D.

Andrea Sawyer-Gray, R.N., P.N.P.-A.C.

Thomas J. Seery, M.D.

S. Kristen Sexson Tejtel, M.D., Ph.D.

Amy Sims, M.D.

Santiago O. Valdes, M.D.

Thomas A. Vargo, M.D.

Giles W. Vick III, M.D., Ph.D.

Steven B. Wolfe, M.D.

Cardiovascular AnesthesiaEmad B. Mossad, M.D., DirectorDean B. Andropoulos, M.D.

Tamra Baker, C.R.N.A.

Beth Barraza, R.N., C.P.N.P.

Kenneth M. Brady, M.D.

Lisa A. Caplan, M.D.

Barbi DeMoss, R.N., C.P.N.P.

R. Blaine Easley, M.D.

Jennifer Esplana, C.R.N.A.

Erin A. Gottlieb, M.D.

Stuart R. Hall, M.D.

Wanda C. Miller-Hance, M.D.

Princy Mohan, R.N., C.P.N.P.

Pablo Motta, M.D.

Laura Oates, C.R.N.A.

Elyse Parchmont, C.R.N.A.

Zoel Quinonez, M.D.

Premal Trivedi, M.D.

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David F. Vener, M.D.

Lauren Weaver, C.R.N.A.

Saeed Yacouby, C.R.N.A.

Jennifer Yborra, R.N., P.N.P.-A.C.

Cardiac Critical CareLara S. Shekerdemian, M.D., F.R.A.C.P.,

M.H.A., ChiefPaul A. Checchia, M.D., F.A.A.P., F.C.C.M.,

F.A.C.C., Medical DirectorNatasha Afonso, M.D.

Marc Anders, M.D.

Patricia Bastero, M.D.

Aarti Bavare, M.D., M.P.H.

Ronald A. Bronicki, M.D., F.C.C.M., F.A.C.C.

Antonio G. Cabrera, M.D.

Heather Chandler, M.D.

Heather A. Dickerson, M.D.

Jordana Goldman, M.D.

Pamela Griffiths, M.D.

Parag Jain, M.D.

Javier Lasa, M.D.

Cesar Mella, M.D.

Elena C. Ocampo, M.D., F.A.A.P., F.A.C.C.

Jack F. Price, M.D.

Asma Razavi, M.D.

Sebastian Tume, M.D.

Travis Vesel, M.D.

Eric A. Williams, M.D., M.S., M.M.M., F.A.A.P.

Developmental OutcomesLara S. Shekerdemian, M.D., F.R.A.C.P., M.H.A.,

Medical DirectorDaniel J. Penny, M.D., Ph.D., M.H.A.,

Medical DirectorLisa Noll, Ph.D.

Eboni Smith, M.D.

Quality Eric A. Williams, M.D., M.S., M.M.M., F.A.A.P.,

Medical DirectorKathleen E. Carberry, R.N., M.P.H.,

Director, Outcomes and Impact Service

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Abuledba K, Cvijanovich NZ, Thomas NJ, Allen GL, Anas N, Bigham MT, Hall M, Freishtat RJ, Sen A, Meyer K,

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Desai MS, Eblimit Z, Thevananther S, Kosters A, Moore DD, Penny DJ, Karpen SJ. Cardiomyopathy reverses

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freestanding children’s hospital. Congenit Heart Dis. 2014; 9:178-186.

Ermis P, Franklin W, Mulukutla V, Parekh D, Ing F. Left ventricular hemodynamic changes and clinical outcomes

after transcatheter atrial septal defect closure in adults. Congenit Heart Dis. 2014.

Ermis PR, Lam WW, Franklin WJ. Pulmonary valvular stenosis in l-transposition of the great arteries with

ventricular septal defect. Tex Heart I J. 2014; 41:240-242.

Ezon DS, Khan MS, Adachi I, Jeewa A, Morris SA, Nagy CZ, Morales DL, Heinle JS. Pediatric ventricular assist

device use as a bridge to transplantation does not affect long-term quality of life. J Thorac Cardiovasc Surg. 2014;

147(4):1334-43.

Fraser CD Jr. Preserving our international heritage of education in congenital heart surgery. J Thorac Cardiovasc Surg. 2014; 148(2):377-8.

Fraser CD Jr. The disadvantaged right ventricle in hypoplastic left heart syndrome: Additional insight. J Thorac Cardiovasc Surg. 2014; 148(5):2419.

Fraser CD Jr. To fenestrate or not: The Fontan debate gets more complicated. J Thorac Cardiovasc Surg. 2014;

148(6):2538-9.

Ghazi P, Moffett BS, Cabrera AG. Hypotension as the etiology for angiotensin-converting enzyme (ace)

inhibitor-associated acute kidney injury in pediatric patients. Pediatr Cardiol. 2014; 35:767-770.

Goldberg JF, Jeewa A, Dreyer WJ, Adams GJ, Cabrera AG, Price JF, Heinle JS, Denfield SW. Postoperative

complications associated with perioperative sirolimus prior to pediatric cardiac retransplantation. J Pediatr Pharmacol Ther. 2014; 19(1):30-4.

Goldberg JF, Mery CM, Griffiths PS, Parekh DR, Welty SE, Bronicki RA, Molossi S. Extracorporeal membrane

oxygenation support in severe hypertrophic obstructive cardiomyopathy associated with persistent

pulmonary hypertension in an infant of a diabetic mother. Circulation. 2014; 18; 130(21):1923-5.

Guzmán-Pruneda FA, Fraser CD Jr. Neuroprotective strategies – what do we really need to know? Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2014; 17(1):77-80.

Hannawi B, Lam WW, Wang S, Younis GA. Current use of fractional flow reserve: A nationwide survey. Tex Heart I J. 2014; 41: 579-584.

Harris BU, Miyake CY, Motonaga KS, Dubin AM. Diagnosis and management of pediatric brugada syndrome: A

survey of pediatric electrophysiologists. Pacing Clin Electrophys. 2014; 37: 638-642.

Hede SV, Morales DL, Parekh DR. At the heart of the old silk road. J Gen Int Med. 2014; 29:1421-1422.

Heinle JS, Gaynor JW. Historical perspectives of The American Association for Thoracic Surgery: John Anton

Waldhausen (1929-2012). J Thorac Cardiovasc Surg. 2014; 148(2):381-6.

Hong BJ, Moffett B, Payne W, Rich S, Ocampo EC, Petit CJ. Impact of postoperative nutrition on weight gain in

infants with hypoplastic left heart syndrome. J Thorac Cardiovasc Surg. 2014; 147:1319-1325.

Ing FF, Khan A, Kobayashi D, Hagler DJ, Forbes TJ. Pulmonary artery stents in the recent era: Immediate and

intermediate follow-up. Catheter Cardiovasc Interv. 2014; 84:1123-1130.

Javois AJ, Rome JJ, Jones TK, Zahn EM, Fleishman CE, Pignatelli RH, Latson LA, Gore HCASG. Results of

the U.S. Food and Drug Administration continued access clinical trial of the gore helex septal occluder for

secundum atrial septal defect. JACC Cardiovasc Interv. 2014; 7:905-912.

Jeewa A, Chin C, Pahl E, Atz AM, Carboni MP, Pruitt E, Naftel DC, Rodriguez R, Dipchand AI, Pediatric Heart

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allograft vasculopathy in pediatric heart transplant recipients: A multi-institutional study. J Heart Lung Transplant. 2014.

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Jeewa A, Manlhiot C, Kantor PF, Mital S, McCrindle BW, Dipchand AI. Risk factors for mortality or delisting of

patients from the pediatric heart transplant waiting list. J Thorac Cardiovasc Surg. 2014; 147:462-468.

Kang KT, Potts JE, Radbill AE, La Page MJ, Papagiannis J, Garnreiter JM, Kubus P, Kantoch MJ, Von Bergen NH,

Fournier A, Cote JM, Paul T, Anderson CC, Cannon BC, Miyake CY, Blaufox AD, Etheridge SP, Sanatani S.

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11:1426-1432.

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Indian experience. Indian J Endocrinol Metab. 2014; 18:13-22.

Kim JJ, Brescia ST, Rossano JW, Pignatelli R, Jefferies JL, Price JF, Decker JA, Denfield SW, Dreyer WJ, Smith

O, Towbin JA. Response to letter regarding article, “Mortality and sudden death in pediatric left ventricular

noncompaction in a tertiary referral center.” Circulation. 2014; 129:e300.

Kong B, Liu Y, Hu H, Wang L, Fan Y, Mei Y, Liu W, Liao J, Liu D, Xing D, Huang H. Left atrial appendage

morphology in patients with atrial fibrillation in china: Implications for stroke risk assessment from a single

center study. Chin Med J. 2014; 127:4210-4214.

Lacro RV, Dietz HC, Sleeper LA, Yetman AT, Bradley TJ, Colan SD, Pearson GD, Selamet Tierney ES, Levine JC,

Atz AM, Benson DW, Braverman AC, Chen S, De Backer J, Gelb BD, Grossfeld PD, Klein GL, Lai WW, Liou A,

Loeys BL, Markham LW, Olson AK, Paridon SM, Pemberton VL, Pierpont ME, Pyeritz RE, Radojewski E, Roman

MJ, Sharkey AM, Stylianou MP, Wechsler SB, Young LT, Mahony L, Pediatric Heart Network I. Atenolol versus

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Lam WW, Patel MB, Rodriguez FH, 3rd, Parekh DR, Franklin WJ. Atriopulmonary fontan spontaneous echo

contrast improved after cardioversion. Tex Heart I J. 2014; 41:100-102.

Lara DA, Lopez KN. Public health research in congenital heart disease. Congenit Heart Dis. 2014; 9: 549-558.

Lawless CE, Asplund C, Asif IM, Courson R, Emery MS, Fuisz A, Kovacs RJ, Lawrence SM, Levine BD, Link MS,

Martinez MW, Matherne GP, Olshansky B, Roberts WO, Salberg L, Vetter VL, Vogel RA, Whitehead J. Protecting

the heart of the American athlete: Proceedings of the American College of Cardiology sports and exercise

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Lawless CE, Olshansky B, Washington RL, Baggish AL, Daniels CJ, Lawrence SM, Sullivan RM, Kovacs RJ, Bove

AA. Sports and exercise cardiology in the United States: Cardiovascular specialists as members of the athlete

healthcare team. J Am Coll Cardiol. 2014; 63:1461-1472.

Lindle KA, Dinh K, Moffett BS, Kyle WB, Montgomery NM, Denfield SD, Knudson JD. Angiotensin-converting

enzyme inhibitor nephrotoxicity in neonates with cardiac disease. Pediatr Cardiol. 2014; 35:499-506.

Lorts A, Zafar F, Adachi I, Morales DL. Mechanical assist devices in neonates and infants. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2014; 17(1):91-5.

Mah DY, Miyake CY, Sherwin ED, Walsh A, Anderson MJ, Western K, Abrams DJ, Alexander ME, Cecchin

F, Walsh EP, Triedman JK. The use of an integrated electroanatomic mapping system and intracardiac

echocardiography to reduce radiation exposure in children and young adults undergoing ablation of

supraventricular tachycardia. Europace. 2014; 16:277-283.

Makari D, Checchia PA, DeVincenzo J. Rationale for full-season dosing for passive antibody prophylaxis of

respiratory syncytial virus. Hum Vaccin Immunother. 2014 Mar; 10(3):604-14.

McKenzie ED, Khan MS, Dietzman TW, Guzmán-Pruneda FA, Samayoa AX, Liou A, Heinle JS, Fraser CD Jr.

Surgical pulmonary valve replacement: A benchmark for outcomes comparisons. J Thorac Cardiovasc Surg. 2014;

148(4):1450-3.

Mery CM, Moffett BS, Khan MS, Zhang W, Guzmán-Pruneda FA, Fraser CD Jr, Cabrera AG. Incidence and

treatment of chylothorax after cardiac surgery in children: Analysis of a large multi-institution database.

J Thorac Cardiovasc Surg. 2014; 147(2):678-86.e1.

Mery CM, Lawrence SM, Krishnamurthy R, Sexson-Tejtel SK, Carberry KE, McKenzie ED, Fraser CD Jr.

Anomalous aortic origin of a coronary artery: Toward a standardized approach. Semin Thorac Cardiovasc Surg.

2014; 26(2):110-22.

Mery CM, Khan MS, Guzmán-Pruneda FA, Verm R, Umakanthan R, Watrin CH, Adachi I, Heinle JS, McKenzie

ED, Fraser CD Jr. Contemporary results of surgical repair of recurrent aortic arch obstruction. Ann Thorac Surg. 2014; 98(1):133-40.

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Mery CM, Guzmán-Pruneda FA, Carberry KE, Watrin CH, McChesney GR, Chan JG, Adachi I, Heinle JS,

McKenzie ED, Fraser CD Jr. Aortic arch advancement for aortic coarctation and hypoplastic aortic arch in

neonates and infants. Ann Thorac Surg. 2014; 98(2):625-33.

Mery CM, Molina KM, Krishnamurthy R, Fraser CD Jr, Justino H. Pulmonary artery resuscitation for isolated

ductal origin of a pulmonary artery. J Thorac Cardiovasc Surg. 2014; 148(5): 2235-2244.e1.

Miyake CY, Teele SA, Chen L, Motonaga KS, Dubin AM, Balasubramanian S, Balise RR, Rosenthal DN, Alexander

ME, Walsh EP, Mah DY. In-hospital arrhythmia development and outcomes in pediatric patients with acute

myocarditis. Am J Cardiol. 2014; 113:535-540.

Moffett BS, Cabrera AG, Teruya J, Bomgaars L. Anticoagulation therapy trends in children supported by

ventricular assist devices: A multi-institutional study. ASAIO J. 2014; 60:211-215.

Moffett BS, Lupo PJ, delaUz CM, Valdes SO, Miyake CY, Decker JA, Kim JJ. Efficacy of digoxin in comparison

with propranolol for treatment of infant supraventricular tachycardia: Analysis of a large, national database.

Cardiol Young. 2014; 1-6.

Moffett BS, Mossad EB, Tobias JD, Cabrera AG. Dexmedetomidine utilisation and outcomes of children with

trisomy 21 undergoing congenital heart disease surgery. Cardiol Young. 2014; 1-5.

Moffett BS, Price JF. National prescribing trends for heart failure medications in children. Congenit Heart Dis. 2015; 10:78-85.

Moffett BS, Syblik D, Denfield S, Altman C, Tejtel-Sexson K. Epidemiology of immunoglobulin resistant

Kawasaki disease: Results from a large, national database. Pediatr Cardiol. 2015; 36:374-378.

Moffett BS, Teruya J. Trends in parenteral direct thrombin inhibitor use in pediatric patients: Analysis of a large

administrative database. Arch Pathol Lab Med. 2014; 138:1229-1232.

Moffett BS, Valdes SO, Lupo PJ, delaUz C, Miyake C, Krenek M, Kim JJ. Flecainide use in children with

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Molina K, Denfield S, Fan Y, Moulik M, Towbin J, Dreyer W, Rossano J. Viral endomyocardial infection in the

1st year post transplant is associated with persistent inflammation in children who have undergone cardiac

transplant. Cardiol Young. 2014; 24:331-336.

Moodie D. On mentoring. Congenit Heart Dis. 2014; 9:169-170.

Moodie D. Long-term follow-up studies in congenital heart disease – how long is long? Congenit Heart Dis. 2014; 9:87-88.

Moodie D. A sit-down with Dr. Denton Cooley. Congenit Heart Dis. 2014; 9:1.

Morris SA, Ethen MK, Penny DJ, Canfield MA, Minard CG, Fixler DE, Nembhard WN. Prenatal diagnosis, birth

location, surgical center, and neonatal mortality in infants with hypoplastic left heart syndrome. Circulation.

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Mulukutla V, Franklin WJ, Villa CR, Morales DL. Surgical device therapy for heart failure in the adult with

congenital heart disease. Heart Fail Clin. 2014; 10:197-206.

Mynard JP, Penny DJ, Smolich JJ. Scalability and in vivo validation of a multi-scale numerical model of the left

coronary circulation. Am J Physiol. 2014; 306:H517-528.

Namachivayam P, Smolich JJ, Shields AE, Rees S, Coleman L, Horton SB, Konstantinov IE, Penny DJ,

Shekerdemian LS. Impact of levosimendan on brain injury patterns in a lamb model of infant cardiopulmonary

bypass. Pediatr Res 2014; 76:64-71.

Nayyar M, Imagawa DK, Tirkes T, Demirjian AN, Houshyar R, Sandrasegaran K, Nangia CS, Seery T, Bhargava P,

Choi JI, Lall C. Composite liver tumors: A radiologic-pathologic correlation. Clin Mol Hepatol. 2014; 20:406-410.

Niu MC, Maskatia SA, Kim JJ. Marked septal dyskinesis from Wolff-Parkinson-White syndrome. Circulation.

2014; 130:e196-198.

Niu MC, Morris SA, Morales DL, Fraser CD Jr, Kim JJ. Low incidence of arrhythmias in the right ventricular

infundibulum sparing approach to tetralogy of Fallot repair. Pediatr Cardiol. 2014; 35(2):261-9.

Noel CV, Kovalchin JP, Adler B, Yates AR. Incidence of tracheobronchial anomalies found with hypoplastic left

heart syndrome. Congenit Heart Dis. 2014; 9:294-299.

Philip J, Burgman C, Bavare A, Akcan-Arikan A, Price JF, Adachi I, Shekerdemian LS. Nature of the underlying

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Pignatelli RH, Texter KM, Denfield SW, Grenier MA, Altman CA, Ayres NA, Chandra-Bose Reddy S. Lv

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Riley A, Sable C, Prasad A, Spurney C, Harahsheh A, Clauss S, Colyer J, Gierdalski M, Johnson A, Pearson GD,

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Seery TJ, Slack MC. Percutaneous closure of a prosthetic pulmonary paravalvular leak. Congenit Heart Dis. 2014;

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Sengupta SP, Amaki M, Bansal M, Fulwani M, Washimkar S, Hofstra L, Narula J, Sengupta PP. Effects of

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Shamszad P, Barnes JN, Morris SA. Aortic dissection in hospitalized children and young adults: A multi-

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Stiller B, Adachi I, Fraser CD Jr. Pediatric ventricular assist devices. Pediatr Crit Care Med. 2013; 14(5 Suppl 1):S20-6.

Taylor B, Moffett BS, Krenek M, Valdes SO, Kim J. Race contributes to beta-blocker efficacy in pediatric

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Thrush PT, Flanigan KM, Mendell JR, Raman SV, Daniels CJ, Allen HD. Visual diagnosis: Chest pain in a boy with

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Toole BJ, Toole LE, Kyle UG, Cabrera AG, Orellana RA, Coss-Bu JA. Perioperative nutritional support and

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Tsang WY, Ziogas A, Lin BS, Seery TE, Karnes W, Stamos MJ, Zell JA. Role of primary tumor resection among

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For more than 50 years, Texas Children’s Heart Center has been saving

lives with the best possible pediatric cardiac care. Our multidisciplinary

team of pediatric cardiologists, cardiovascular surgeons, cardiovascular

anesthesiologists, perfusionists, adult congenital heart specialists, cardiac

intensivists, nurses, child life specialists and support staff work together

to surround patients with expertise at every point from diagnosis through

treatment in order to provide the best possible results.

Meet the Texas Children’s Heart Center Team.( F L I P O P E N )

Wander GS, Kapoor R, Bansal M, Ahlawat K, Trehan N. Iatrogenic large intramyocardial pseudoaneurysm after

coronary artery bypass surgery and its successful percutaneous closure. J Am Coll Cardiol. 2014; 63:e47.

Wang G, Knight L, Ji R, Lawrence P, Kanaan U, Li L, Das A, Cui B, Zou W, Penny DJ, Fan Y. Early onset severe

pulmonary arterial hypertension with ‘two-hit’ digenic mutations in both bmpr2 and kcna5 genes. Int J Cardiol. 2014; 177:e167-169.

Wang H, Ji R, Meng J, Cui Q, Zou W, Li L, Wang G, Sun L, Li Z, Huo L, Fan Y, Penny DJ. Functional changes in

pulmonary arterial endothelial cells associated with bmpr2 mutations. PloS One. 2014; 9:e106703.

Weiss SL, Cvijanovich NZ, Allen GL, Thomas NJ, Freishtat RJ, Anas N, Meyer K, Checchia PA, Shanley TP,

Bigham MT, Fitzgerald J, Banschbach S, Beckman E, Howard K, Harmon K, Wong HR. Differential expression

of the nuclear-encoded mitochondrial transcriptome in pediatric septic shock. Critical Care. 2014 Nov 19;

18(6):623. [Epub ahead of print]

Wingate JR, Adachi I, Mossad EB. Case 14 – 2014: Tetralogy of Fallot with severe cyanosis in an infant with

trisomy 18: Ethical dilemmas in the perioperative period. J Cardiothorac Vasc Anesth. 2014; 28(6):1677-85.

Wong HR, Weiss SL, Giuliano JS, Wainwright MS, Cvijanovich NZ, Thomas NJ, Allen GL, Anas N, Bigham MT,

Hall M, Freishtat RJ, Sen A, Meyer K, Checchia PA, Shanley TP, Nowak J, Quasney M, Chopra A, Fitzgerald JC,

Gedeit R, Banschbach S, Beckman E, Harmon K, Lahni P, Lindsell CJ. The temporal version of the pediatric

sepsis biomarker risk model. PLoS One. 2014 Mar 13; 9(3):e92121.

Wong HR, Weiss SL, Giuliano JS, Wainwright MS, Cvijanovich NZ, Thomas NJ, Allen GL, Anas N, Bigham MT,

Hall M, Freishtat RJ, Sen A, Meyer K, Checchia PA, Shanley TP, Nowak J, Quasney M, Chopra A, Fitzgerald JC,

Gedeit R, Banschbach S, Beckman E, Harmon K, Lahni P, Lindsell CJ. Testing the prognostic accuracy of the

pediatric sepsis biomarker risk model. PLoS One. 2014 Jan 29; 9(1):e86242.

Wong HR, Cvijanovich NZ, Allen GL, Thomas NJ, Freishtat RJ, Anas N, Meyer K, Checchia PA, Weiss SL,

Shanley TP, Bigham MT, Banschbach S, Beckman E, Harmon K, Zimmerman JJ. Corticosteroids are associated

with repression of adaptive immunity gene programs in pediatric septic shock. Am J Respir Crit Care Med. 2014

Apr 15; 189(8):940-6.

Yu X, Cao X, Chen X, Ayres N, Zhang P. Triplet-triplet annihilation upconversion from rationally designed

polymeric emitters with tunable inter-chromophore distances. Chem. Commun. 2015; 51:588-591.

Zdradzinski MJ, Qureshi AM, Stewart R, Pettersson G, Krasuski RA. Comparison of long-term postoperative

sequelae in patients with tetralogy of fallot versus isolated pulmonic stenosis. Am J Cardiol. 2014; 114:300-304.

Zhang L, Mmagu O, Liu L, Li D, Fan Y, Baranchuk A, Kowey PR. Hypertrophic cardiomyopathy: Can the

noninvasive diagnostic testing identify high risk patients? World J Cardiol. 2014; 6:764-770.

Texas Children’s Heart Center ReferralsTo refer your pediatric patient to Texas Children’s Heart Center, please call 832-82-HEART

(832-824-3278).

Congenital Heart Surgery The Congenital Heart Surgery service at Texas Children’s Hospital offers same-day surgical

consultation appointments. To make an appointment for a same-day consultation, call

832-TCH-APPT (832-824-2778). For additional appointment information or to speak

with a division administrator, please call 832-826-2030.

Cardiology, Adult Congenital Heart Disease and the Cardiovascular Intensive Care Unit 832-82-HEART (832-824-3278)

Heart Transplant Program 866-683-8032

Cardiovascular Anesthesia 832-826-5831

Visit texaschildrens.org/refer for more information about referring a patient, or learn more

about Texas Children’s Heart Center at texaschildrens.org/heart.

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Texas Children’s Heart Center