UNC Children's Heart Center A New Path Forward by UNC Surgery

UNC Children's Heart Center UNC Children's Heart Center A New Path Forward A New Path Forward

Welcome

We are honored to be the co-directors of The University of North Carolina Children's Heart Center. The Heart Center is not just a base for clinical and research activity, but our home. We are privileged to work with a dedicated team of faculty and staff whose daily priority is assisting us in our mission of providing holistic, family-centered care. Our singular focus, to provide exemplary care through research, teaching, and clinical innovation, is the cornerstone of The Heart Center. North Carolina Memorial Hospital has been providing heart care to the citizens of our state for over 60 years. The parallel developments in pediatric cardiac surgery and cardiology led to the rapid expansion of medical services and growth at The University of North Carolina at Chapel Hill. As such, the Children’s Heart Center was established within UNC Children’s Hospital. As the state's hospital, we provide specialty care to more than 70,000 children from all 100 counties in North Carolina each year, and we are recognized by U.S. News & World Report as one of "America's Best Children's Hospitals" on its annual list. The program offers comprehensive services for patients with congenital heart disease from prior to birth through adulthood in a family-centered, holistic fashion. Our mission is to provide care to all of the state's citizens regardless of geographic or financial circumstances. The multidisciplinary care team includes specialists from pediatric cardiology, congenital heart surgery, anesthesiology, maternal-fetal medicine, neonatology, cardiac critical care, and nursing along with dedicated providers in social work, pharmacy, and nutrition. The Heart Center has a strong emphasis on clinical care, but significant effort is also devoted to education and research. We are fortunate to have department, university, and hospital leadership support our many transformative changes focused on high-quality care with an emphasis on patient value. It is our pleasure to be part of this outstanding team, and we are humbled to be surrounded by our world-class colleagues and dedicated faculty and staff. We hope you will use the information included in this compendium as a resource to get to know us and provide an overview of the depth of services we provide here at The University of North Carolina Medical Center. Sincerely,

Timothy M. Hoffman, MD Chief, Pediatric Cardiology Co-Director of UNC Children's Heart Center

Mahesh S. Sharma, MD Chief, Congenital Cardiac Surgery Co-Director of UNC Children's Heart Center

MISSION To promote the well-being of all patients with congenital and acquired heart disease through high-quality, comprehensive care from fetus through adulthood.

VISION To be a recognized leader of excellence in familycentered, comprehensive cardiac care.

Mahesh S. Sharma, MD, FACS serves

FACC is the Co-Director of the UNC

as Chief of Congenital Cardiac Surgery

Children’s Heart Center, Division

and Co-Director of UNC Children’s

Chief of Pediatric Cardiology, and

Heart Center, as well as Associate

Governors Club Distinguished

Professor of Surgery and Pediatrics

Professor in Pediatric Cardiology

at the UNC School of Medicine. In

at The University of North

addition, he is the director of pediatric

Carolina at Chapel Hill. He earned

heart transplantation and mechanical

his medical degree at the West

circulatory support and surgical

Virginia University School of Medicine in Morgantown,

director of the UNC Adult Congenital Heart Program. Dr.

WV, where he also completed residency in pediatrics. His

Sharma graduated with honors from The University of Texas

postgraduate training included a fellowship in pediatric

at Austin and received his medical doctorate from Baylor

cardiology at The Children's Hospital of Philadelphia,

College of Medicine in Houston.

followed by a fellowship in cardiac critical care and thoracic organ transplantation.

His postdoctoral education includes general surgery residency at Tufts University School of Medicine and

Dr. Hoffman joined the faculty at The University of

cardiothoracic surgical training at The University of

Pennsylvania School of Medicine as Assistant Professor

Pittsburgh and Children’s Hospital of Pittsburgh. He

of Pediatrics, Medical Director of Thoracic Organ

subsequently completed a fellowship in pediatric

Transplantation, and a member of the section of cardiac

cardiothoracic surgery and cardiopulmonary transplantation

critical care at The Children's Hospital of Philadelphia.

at the Great Ormond Hospital for Children in London,

Subsequently, he became the Medical Director of the Heart Transplant and Heart Failure Program at Nationwide Children's Hospital, Associate Medical Director of Cardiology, Pediatric Cardiology Fellowship Program

England, one of Europe’s leading congenital heart centers. Dr. Sharma joined the faculty at the University of Texas Southwestern Medical Center as Assistant Professor of Surgery

Director and Professor of Pediatrics, Division of Cardiology

and attending surgeon at Children’s Medical Center Dallas. He

at The Ohio State University College of Medicine until 2015

then returned to The University of Pittsburgh and Children’s

when he joined UNC.

Hospital of Pittsburgh as Assistant Professor of Surgery and

Dr. Hoffman served for five years as the pediatric liaison to the American Heart Association (AHA) Heart Transplant and Heart Failure Committee and was a member of the inaugural AHA Cardiovascular Disease in the Young Heart Failure Committee. Dr. Hoffman is involved in several multicenter research collaborations focusing on heart

Director of Pediatric Mechanical Circulatory Support and Artificial Heart Technology. During his tenure, both centers were ranked among the best programs in the nation by US News & World Report. In 2018, Dr. Sharma was recruited to the University of North Carolina at Chapel Hill to help direct surgical services in the UNC Children’s Heart Center.

transplant care, treatment of the failing myocardium, and

Dr. Sharma is the author of over 100 peer review abstracts,

cardiac support in the peri-operative period. He also is an

journal articles, and book chapters. He is a Diplomat of

active participant and co-author of the Pediatric Heart

the American Board of Thoracic Surgery with special

Transplant Study Group. Dr. Hoffman also served on the

certification in congenital heart surgery and a member of

Board of Directors for the Pediatric Cardiac Intensive Care

numerous professional societies. He currently serves on

Society and received the Presidential Award for Service

the Society for Thoracic Surgeons Workforce on Congenital

in 2014. He currently serves on the American Board of

Heart Surgery. Dr. Sharma has been named one of the

Pediatrics Sub-board of Pediatric Cardiology. He has been

nation’s top doctors by US News & World Report in

listed in Best Doctors since 2003.

partnership with Castle Connolly Medical, Ltd.

Heart Center Leadership

HEART CENTER LEADERSHIP

Timothy M. Hoffman, MD, FAHA,

UNC MaternalFetal Medicine UNC Maternal-Fetal Medicine (UNC MFM) and the UNC Fetal Care Center is proud to work in collaboration with the UNC Children’s Heart Center to care for families with the diagnosis of a fetal cardiac condition. Complications in pregnancy can arise due to maternal health conditions or can occur due to the development of obstetric conditions such as preterm labor or preeclampsia. Maternal-fetal medicine providers most often work in conjunction with the patient’s primary obstetrics provider to assist in management of specific issues or may provide complete prenatal and delivery care. The UNC MFM team includes subspecialty certified high-risk pregnancy physicians and providers, reproductive genetics services, dedicated MFM sonographers with fetal echocardiography certification and comprehensive care coordination. The MFM team will help determine the type of cardiac condition as well as ensure the overall health of the mother and baby and work with Heart Center providers to develop a plan of care for a healthy pregnancy, including fetal monitoring and delivery planning. Additionally, the MFM team provides advanced diagnostic testing such as dedicated MFM ultrasound imaging, amniocentesis, fetal MRI and genetic diagnosis to ensure the optimal pregnancy care plan. MFM management may include prenatal care visits and regular fetal monitoring with ultrasound as well as inpatient management and maternal treatment for selected conditions. Delivery at UNC will be coordinated by the MFM team. The Center for Maternal and Infant Health provides care coordination services for the family during the pregnancy and into infancy. 6

Fetal Imaging The Fetal Cardiology Program at UNC has provided comprehensive care to families in North Carolina and the Southeast for over 25 years. The dedicated fetal cardiac imaging team includes pediatric sonographers who are board certified in fetal echocardiography and cardiologists dedicated to fetal imaging. The group provides diagnosis, in utero therapy, counseling, and delivery planning to expectant families in our multiple locations throughout the state. Our goal is always to keep families close to home, and we work directly with our partner obstetricians, neonatologists, and hospitals to deliver locally if possible. The Fetal Cardiology Program is integrated with the UNC Center for Maternal and Infant Health, which provides care coordination to families with high-risk pregnancies. These families have a personal care coordinator who gathers records, schedules appointments, gives tours and is a resource for all issues related to the pregnancy. All cases are discussed in a weekly interdisciplinary case conference where all the providers who will be taking care of both the mother and the baby are involved. A personalized care management plan for the family is developed prenatally, and both the mother and baby are followed in the Center through discharge or longer to ensure continuity of subspecialty care at UNC and reintegration back into the community with specialized resources as needed.

PATIENT STORY TANNER Therapies for Hypoplastic Left Heart Syndrome (HLHS) Tanner was born with HLHS, a rare heart malformation in which the main pumping chamber of the heart (left ventricle) is severely underdeveloped and not capable of providing oxygen-rich blood to his organs. If untreated as a newborn, it is uniformly fatal. Tanner underwent a series of staged palliations (operations) starting shortly after birth, including a high-risk and complex procedure called the Norwood operation in which his right pumping chamber was connected to his main blood vessel (aorta) allowing for his left heart to be “bypassed” from his circulation while enlarging the aorta. Without a pump to his lungs, UNC surgeons connected a small tube or shunt from his right ventricle to his lung arteries creating a system where the single pump does the work of both sides of the heart. One of the most challenging conditions to treat, UNC Children’s Heart Center has had tremendous success with this approach in recent years. Tanner progressed to Stage II palliation in which his right heart was separated from his veins allowing “blue” blood to travel directly to his lungs without a pump, known as a cavopulmonary connection or bidirectional Glenn procedure. Despite these major challenges, Tanner is thriving, happy and healthy as he awaits completion of his single ventricle pathway. 7

Neonatology The Neonatal Critical Care Center at the University of North Carolina Children’s Hospital is a 58-bed facility served by 13 board-certified/eligible attending neonatologists, 23 nurse practitioners, 7 fellows and more than 150 neonatal specialists from UNC Hospitals. The Center averages 800 admissions annually, providing care to newborns from over 50 counties in North Carolina. In collaboration with specialized nurses, respiratory therapists, pharmacists, nutritionists, rehabilitation therapists, social workers and pediatric subspecialists, physicians and neonatal nurse practitioners provide care for infants with birth defects, preterm birth, and a wide variety of other medical and surgical conditions. After discharge, infants who are at risk for neurodevelopmental impairments, such as those with preterm birth, congenital heart disease or neonatal encephalopathy, are evaluated in the Special Infant Care Clinic, a subspecialty outpatient clinic designed to assist community health care providers and promote the best developmental outcomes for these children. The clinic offers a multidisciplinary approach to the developmental follow-up of high-risk infants from birth to 2 years of age, complementing the health care provided in the local community. 8

Cardiac Imaging The diagnostic capabilities of cardiac magnetic resonance imaging (CMR) and computed tomography angiography (CTA) are increasingly being used to replace more invasive tests for many patients. The Heart Center cardiac imaging team uses cardiac MRI, particularly in older children and adolescents, to gather valuable information, including cardiac muscle characteristics, cardiac performance, and blood flow through the heart’s vessels. The process is built on a strong collaboration between pediatric cardiology and pediatric radiology, bringing the unique expertise from each field to care for children. Pediatric trained nurses, radiology technicians, and in some circumstances, pediatric anesthetists are also essential in obtaining these studies. The cardiac imaging team performs cardiac CTA (often known as “CAT scans”) routinely to obtain detailed anatomy information, particularly in infants and young children. These scans (example pictured to the right) similarly require a team-based approach, and there will typically be a cardiologist, radiologist or both at the scanner, participating in acquiring the child’s images. The team uses a third-generation dual-source multidetector CT scanner (Siemens SOMATOM Force). Besides being thoughtful about precisely what information is needed, technological advancements minimize children’s radiation exposure. The imaging team then reads the study collaboratively. They work with a dedicated 3D post-processing lab to make 3D models of the child’s anatomy. This enhances a review of the child’s anatomy during combined cardiology and cardiothoracic surgery case management conference should that be necessary. Additionally, the processing lab has full 3D printing capabilities, which can provide cardiothoracic surgical and interventional cardiology teams with models to plan surgical or procedural interventions. Time is valuable if a child ultimately requires an operation or procedure.

PATIENT STORY SUHA Complex Pulmonary Atresia and The “Unifocalization” Procedure Suha has a rare form of Tetralogy of Fallot in which her right heart never made a connection to her lung arteries during development. As a result, her lungs received blood flow from numerous collateral vessels called “MAPCAs” from her aorta. Suha’s parents were told by doctors in her native country, that at that time, there were no treatments available to save her. Conditions such as Suha’s are universally fatal if not treated because children become progressively “blue” or cyanotic and rarely live to adulthood. Fortunately, she defied the odds, and her family relocated to the United States. She found her way to North Carolina and the UNC Adult Congenital Heart Program. Dr. Michael Yeung assessed Suha along with the Adult Congenital Heart Team. Advanced 3D imaging (image shown) and cardiac catheterization were required to create a “road map” so our surgeons could divide or “septate” her heart. This involved placing a patch inside while bringing the many collaterals together to create a new lung artery confluence to accept blood from her right heart using a valved tube or conduit from a human donor. The so-called “unifocalization” procedure is a complex and intricate operation performed in only a select number of centers around the country and even fewer who take patients nearing end-stage disease as adults to complete this repair. Suha had an excellent recovery and for the first time in her life, her skin and lips were pink instead of blue from cyanosis or lack of oxygen in her blood. 9

Interventional Catheterization The Interventional Cardiac Catheterization Program at the UNC Children's Hospital provides a broad range of minimally invasive catheterbased treatments for patients of all ages with congenital heart disease. The program has a legacy of participation in clinical trials for catheterdirected implants leading to FDA approval and widespread adoption of techniques now accepted as standard of care. Our program performs the full breadth of standard procedures—balloon valvuloplasty and angioplasty, intravascular stent implantation, transcatheter closure of congenital shunts (atrial and selected ventricular septal defects, patent ductus arteriosus), and transcatheter valve implantation. In some patients, newer techniques are applied, such as catheter closure of the large patent ductus arteriosus in tiny, fragile premature infants, stenting of the ductus arteriosus for palliation of pulmonary blood flow, and transcatheter pulmonary valve implantation in adults without prior surgical conduit. The pediatric cardiac catheterization suite is a recently updated, self-contained care area adjoining the UNC Children's Heart Center. The catheterization laboratory houses modern biplane angiography equipment that supports adjunctive advanced imaging guidance and minimizes radiation dose. The suite is staffed by allocated pediatric nursing personnel with expertise in caring for patients with congenital heart disease and their families, as well as coordinating care with the hospital-based care team when appropriate. Procedures are supported by pediatric cardiac anesthesiology staff to ensure optimal safety and comfort. 10

The Interventional Congenital Catheterization Program performs several hundred procedures annually across all ages and maintains an excellent track record of safety and quality outcomes. The program participates in the American College of Cardiology’s IMPACT registry of quality surveillance.

Electrophysiology The Electrophysiology Program at UNC Children's Heart Center provides comprehensive care for patients with heart rhythm abnormalities. Heart rhythm abnormalities can begin before birth or develop throughout childhood; they can be in isolation or in conjunction with structural congenital heart disease; they can be transient problems or multi-generational inherited lifelong concerns.

The resurgence of the Congenital Heart Program has been fostered by the multidisciplinary team members who are the

Physicians use diagnostic and therapeutic modalities to care for children with heart rhythm abnormalities. Electrocardiography, extended ambulatory heart rhythm monitoring and exercise testing are the primary noninvasive tools used. For some children, long-term implanted loop recorders provide additional information not otherwise obtainable. Invasive intracardiac electrophysiology testing is performed using fluoroscopic (X-ray) and non-fluoroscopic mapping technologies, with ablation procedures performed using radiofrequency or cryoablation catheter techniques, depending on the type of arrhythmia. Cardiac pacemakers and defibrillators present unique opportunities and challenges in children. Young children too small for "adult style" transvenous devices as well as older patients whose anatomy precludes traditional device and lead location particularly benefit from collaboration between surgeons and pediatric electrophysiologists. This teamwork optimizes implant success, device programming, and long-term outcome. Similar individualized, collaborative care is provided to older congenital heart disease pacemaker/defibrillator patients as they transition

pillars of UNC Children’s Heart Center. The program is rapidly becoming one of the premier pediatric cardiac surgery programs in the region.

to the UNC Adult Congenital Heart Disease Program. 11

PATIENT STORY

The Heart Failure and Heart Transplant Program at the UNC

GRACIE

Children’s Heart Center is a comprehensive service that provides

Left Ventricular Assist Device (LVAD)

across the state of North Carolina. This service combines

global care for children of all ages. The program continues to grow, providing care to families in many regional clinics

Gracie was enjoying life as a teen until she

expertise in outpatient and inpatient care with noninvasive

developed sudden shortness of breath and

imaging, including the latest in MRI techniques, catheterization,

malaise. She was transferred to UNC after an

electrophysiology, device therapy, and adult congenital and

X-ray showed an enlarged heart. Tests confirmed

acquired heart disease care.

the diagnosis of dilated cardiomyopathy, a rare disease of the heart muscle that causes the heart to deteriorate to the point of failure. She was supported with medications in consult with our pediatric heart failure team. Gracie continued to have symptoms including intestinal angina, a condition where her bowel did not receive

Under the leadership of Drs. Timothy Hoffman, medical director, and Mahesh Sharma, surgical director, the multidisciplinary team includes nurse coordinators, social workers, pharmacists, nutritionists, child life specialists, and many others. The team also engages a broad range of subspecialist physicians involved in the care of children afflicted with heart failure.

enough oxygenated blood, leading to severe

The program’s success hinges on how we deliver care to the

abdominal pain. The team recognized she was

patients and their families through a unique physician-nurse

rapidly declining and recommended a state-of-

model. This model includes a service-oriented and timely

the-art technology called an LVAD, or artificial

response program assuring patients’ needs are met. As the

heart, to support her circulation while she was

field of mechanical circulatory assistance for children evolves,

listed for heart transplantation.

our program employs the latest in artificial heart technology,

Our surgeons implanted a pump into Gracie’s heart

including Berlin Heart EXCOR®, PediMag and HeartMate3

that took over function of her weakened heart and

LVADs. Also, we have specialized providers and miniaturized

allowed her to rehabilitate. She was the first child

technology for the short-term support of the failing heart and

at UNC to receive such lifesaving technology and

lungs using extracorporeal membrane oxygenation.

still be able to leave the hospital while she waited for her new heart. Several months later, when she was back to full activity at home with her LVAD, the team received a call about a suitable donor. Gracie was admitted and underwent successful heart transplantation. She’s had an uneventful recovery and was discharged home. She’s now back to living a full life and has regained her spunky personality. 12

Therapies for End-Stage Heart Failure

Our program has participated in several registries but most notably with the Pediatric Heart Transplant Society (PHTS). The PHTS study registry was established in 1993, and our program has participated for decades. This registry has advanced the understanding and care surrounding pediatric heart transplantation. The program has received recognition through our national leadership including invited lecturships and multicenter research initiatives.

Congenital Heart Surgery With the advent of the heart-lung machine and early pioneering efforts of Dr. Benson R. Wilcox, pediatric open-heart surgery at UNC was established on April 3, 1957. The Section of Congenital Cardiac Surgery is a leading provider of comprehensive congenital cardiovascular services in North Carolina. Our boardcertified congenital heart surgeons have a proven record in performing procedures considered cuttingedge, including surgeries for low-birth-weight babies, surgery for hypoplastic left heart syndrome, airway surgery and highly specialized treatment for pulmonary atresia with major aortopulmonary collateral (the socalled “unifocalization” procedure). Additionally, our surgeons have rare and unique experience in the surgical management of complex pediatric valve lesions, including aortic valve repair and surgical therapy for Ebstein’s anomaly (the DaSilva Cone Procedure). We have a nationally recognized center to treat pediatric heart failure, cardiomyopathy, heart and lung transplantation, and mechanical circulatory support (artificial heart technology). We are one of the few centers in the nation providing access to the full spectrum of pediatric and adult congenital cardiopulmonary transplant options. In addition, UNC is one of a limited number of facilities in the region with a dedicated pediatric airway center and the only one in the state of North Carolina. Our surgeons perform complex airway reconstructions, including slide tracheoplasty procedures for long-segment tracheal stenosis. An ongoing program of parent and patient education about congenital heart surgery is overseen by pediatric advanced nurse practitioners and physician assistants whose sole job is to care for these children with complex conditions. A dedicated pediatric cardiac social worker also serves as a compassionate liaison among families, physicians and other hospital personnel. 13

Results That Matter The ultimate test for teams who provide care for the most critically ill patients afflicted with congenital heart disease is the overall procedural outcomes. Our center publicly reports our outcomes data through the Society for Thoracic Surgeons (STS) to maintain full transparency and to help parents select the best possible care. Congenital heart operations are grouped by the complexity of the procedure in what is known as STAT Categories. Our current mortality rate for all index cardiac surgical procedures among all STAT Categories is 2.3%. This means that of all children operated on at UNC from June 2018 to July 2021, including those with less complicated heart conditions to those with the most severe forms of congenital heart disease, 98% of our patients survived their procedures and were able to leave the hospital. Not only is this the highest survival rate for congenital heart surgery in our program’s history, but the outcomes are also better than the national average. Our team was able to achieve a 100% post-transplant survival rate in pediatric patients. During the same time period, we have performed an unprecedented number of pediatric heart transplants. This is among the best transplant survival rates in the nation and better than the national average.*

Par ticipant 2021

2.3%

Our neonatal, infant, pediatric, and adult congenital cardiac surgery (STAT 1-5) overall STS index mortality

100% post-transplant survival rate in pediatric patients June 2018 to July 2021

Congenital Heart Surgery Data Summary Mortality Data

DEANNA

3.0 2.9

2.7%

2.8 2.7 2.6 2.5 2.4 2.3

2.3%

2.2 2.1 2.0 1.9

UNC data from 2018-2021 as compared to STS Congenital Heart Surgery Data Summary Spring 2019 Harvest.

Heart Transplant Survival 105 100 95 90

PATIENT STORY

100% 92.9% 86.5%

Anomalous Aortic Origin of the Coronary Artery (AAOCA) Deanna was troubled by a recurrent pain in her chest. The mother of three thought she may have had a bad case of reflux or indigestion. She was referred by her doctor after having an abnormal stress test, and a heart ultrasound suggested an abnormality of her coronary artery, or artery to her heart muscle, that was concerning. The newly formed Coronary Anomalies Program within the UNC Adult Congenital Heart Program allowed Deanna to receive comprehensive multidisciplinary assessment and timely surgical care. This prompt attention prevented the most dreaded complication from her illness—“sudden death” due to lack of blood to her heart muscle and potential for irregular heartbeats. Deanna underwent a successful coronary “unroofing” procedure to create a wide open channel for blood to flow to her heart. She recovered, and her follow-up testing revealed the artery is widely open and she has no signs of limited blood flow to the area of heart muscle previously in jeopardy. She has resumed a very active life and enjoys spending time with her family.

80 75

Expected Survival

United Network for Organ Sharing National Benchmark from 2019 Annual Data Report of the Scientific Registry of Transplant Recipients (SRTR). 15

Cardiovascular Intensive Care Unit The Pediatric Cardiac Intensive Care Unit (PCICU) is a dedicated 10-bed unit at the North Carolina Children’s Hospital. The PCICU is a vibrant team of cardiac intensivists, critical care fellows, anesthesiologists, pharmacists, advanced practice providers, nurses, respiratory therapists, social workers, dietitians and support staff.

Teamwork is a crucial aspect of care in the Pediatric Cardiac Intensive Care Unit

This highly specialized team provides care for the full spectrum of patients with congenital heart disease, from newborns to young adults. It is the focal point and exemplar for multidisciplinary care, with cardiologists and cardiac surgeons participating daily in bedside rounds with the PCICU team. The PCICU is also the home of advanced care for end-stage heart disease and mechanical circulatory support, including extracorporeal membrane oxygenation and ventricular assist device technologies. All PCICU faculty are trained in pediatric critical care medicine and have, combined, over 50 years of experience caring for critically ill pediatric patients with heart disease. In 2019, the department added experienced faculty who have completed training in pediatric cardiology and cardiac critical care. 16

To foster positive team dynamics, the PCICU uses simulation to bring together team members with different roles and backgrounds. The simulation cases take place within the PCICU to ensure high fidelity and are based on real experiences targeting education for both medical knowledge and communication among the participants.

PATIENT STORY ASAEL Orthotopic Heart Transplantation Asael is a young boy who suffers from Gitelman’s syndrome, a rare genetic disorder in which there is a specific defect in kidney function. The abnormality was first discovered in 1966 by nephrologist Hillel J. Gitelman, MD while working at UNC. This defect impairs the kidney’s ability to reabsorb salt and leads to imbalances in electrolytes. Many of these electrolytes are important in heart function, and some children develop a weakening of the heart muscle leading to heart failure. Asael’s heart was so weak he required support from a ventilator and developed a stroke. UNC Children’s Heart Center doctors treated and cared for his medical problems while he recovered, and he was removed from life support. His heart did not recover, and he needed a replacement. Asael was placed on the transplant list awaiting a suitable donor. He was successfully transplanted and is one of the very few patients in the world with this condition who has undergone pediatric heart transplantation for cardiomyopathy related to his underlying kidney disorder. Asael was discharged from the hospital with excellent heart function and has made a full recovery. Our team recently published this incredible work in the medical literature (Solt SA, Hoffman TM, Sharma MS, et al. Orthotopic Heart Transplantation in a Patient With Gitelman Syndrome and Dilated Cardiomyopathy. World Journal of Pediatric and Congenital Heart Surgery, Volume 11, issue: 4, pp. 520-521.) 18 6

Nursing Our nurses are an integral part of the UNC Children’s Heart Center multidisciplinary team and work in a variety of areas of expertise to contribute to the delivery of exceptional patient care. The nursing team is composed of dedicated professionals committed to patients and their families throughout the Heart Center. They offer specialized nursing care of infants, children and young adults with congenital heart disease and other cardiac diagnoses. Our highly skilled staff offers a compassionate, family-centered approach to providing the children of North Carolina with the best care.

Our committed staff includes specialty trainedcardiac nurses who not only provide exceptional care at the bedside, but also lead cardiac-specific quality improvement projects and education initiatives and actively participate in the Family Advisory Council. UNC Health’s Department of Nursing was redesignated for the third time, as a Magnet institution by the American Nurses Credentialing Center Magnet Recognition Program in 2020. UNC Health is part of this select group of <9% of health care organizations nationwide demonstrating continued improvements in quality and patient care.

Adult Congenital Heart Program The UNC Adult Congenital Heart Disease Program provides specialized care for adults who are living with congenital heart disease. Our medical staff includes specially trained board-certified cardiologists, cardiovascular surgeons, advanced practice providers, nurses, and technicians. The multidisciplinary team evaluates and develops a personalized treatment plan for patients who need monitoring throughout their lifetime. Our outpatient services are housed in our stateof-the-art clinic in the UNC Hospitals Heart and Vascular Center at Eastowne. Our team offers a broad spectrum of treatments including surgery and interventions for conditions such as anomalous aortic origin of the coronary artery (AAOCA), less invasive treatments for valvular heart disease including transcather valve replacement, novel valve repair treatments including aortic valve repair (Ozaki procedure), tricuspid valve repair (DaSilva Cone procedure), and pulmonary autograft (Ross procedure). We are one of a very select centers in the country offering comprehensive options for valvular heart disease. Additional therapies include closure of septal defects, repair of anomalous pulmonary venous return, pacemaker/defibrillator implantation, repair of coarctation of the aorta, single ventricle palliation/Fontan conversion, surgery for Tetralogy of Fallot including aortopulmonary collaterals, and the medical and surgical treatment of end-stage heart failure.

Cardiovascular Anesthesia The pediatric cardiac anesthesiology team team at UNC Children’s Hospital provides sedation and anesthesia services for all congenital cardiac operations and children with complex congenital heart disease undergoing various non-cardiac surgeries and procedures. Our team of board-certified pediatric anesthesiologists provide specialized anesthesia care for cardiac catheterization and electrophysiology procedures as well as imaging studies and interventional radiology.

PATIENT STORY BILL Options for Aortic Valve Disease—The Pulmonary Autograft (Ross) Procedure Bill is a healthy and energetic young man with a zest for life. He was diagnosed as a child with an abnormal heart valve that restricted the amount of blood leaving his heart to his brain, kidneys, and other vital organs. As time went on, Bill became more fatigued and short of breath. His heart was working double-time, and the muscle was beginning to become thickened or hypertrophied. This condition eventually would have led to heart failure or a heart attack. Bill sought care with our Adult Congenital Heart Team and discussed options for treatment. Due to Bill’s active lifestyle, a valve replacement requiring any blood thinners was not ideal for him. Thus, he researched other options including the Ross procedure. Expertise in this complex operation is limited, and Bill reached out to a number of centers around the country as well as UNC where surgeons have special expertise and a track record for performing this surgery. Our adult congenital heart surgeons performed a modification for this operation. Bill’s pulmonary valve was removed (harvested) from his heart and placed within a cylinder to prevent his pulmonary valve from bulging out under high pressure, and this cylinder was used to replace Bill’s diseased aortic valve. Our surgeons also repaired an aneurysm (ballooning) of Bill’s main artery from his left heart to reduce the risk for rupture. Bill had an excellent recovery and has his native pulmonary valve working in his aorta. This avoids blood thinners and an artificial aortic valve for potentially the remainder of his life. 19

Quality Program UNC Childrens’s Heart Center and the Adult Congenital Heart Program are committed to ongoing quality improvement as a mechanism to optimize care for the patients we serve. As such, we have a strong tradition of dedicated quality initiatives.

EDIATRIC ARDIAC RITICAL ARE ONSORTIUM

The Teamwork to Improve Cardiac Kids’ End Results (TICKER) Program began in 2010 as an Agency for

4 Improving outcomes and

Healthcare Research and Quality grant-funded project aimed to improve teamwork and standardize care for pediatric congenital heart disease patients. Our multidisciplinary team also focused on familycentered care for the parents of a child with congenital heart disease cared for at UNC Children’s. This team set the foundation and infrastructure for the mindset of continuous quality improvement and growth for our pediatric cardiac program. In recent years, working with the UNC Children’s Improvement Council, the hospital leadership has emphasized the prioritization of continual hospitalwide improvement. In 2016, a Pediatric Cardiac Improvement Team formed to continue the efforts of the Project TICKER team. Each year, guided by an in-depth comparison of the Society for Thoracic Surgery data, this multidisciplinary improvement team focuses on a specific area of care for a comprehensive improvement project. The positive outcomes from these projects have been presented at national conferences. By working to standardize care, foster communication among multiple teams and provide ongoing education for our faculty, staff and families, we hope to continue to deliver on our promise of exemplary family-centered cardiovascular care.

quality through collaboration

Research The pursuit of new knowledge through basic, clinical and translational research leads to new treatments and improvements in care for patients with congenital and acquired heart disease. In addition, our center’s participation in important registries such as the STS Congenital Heart Surgery Database (CHSD), Improving Pediatric and Adult Congenital Treatment Registry (IMPACT) and anticipated inclusion in the Pediatric Cardiac Critical Care Consortium Registry (PC4) helps move the field forward in a collaborative fashion with other centers around the world, so large amounts of data can be distilled down to meaningful gains for our patients. Our noninvasive laboratory serves as a core facility for many ongoing single-center and multicenter clinical trials that encompass different disciplines, including cardiology and cardiothoracic surgery. Our faculty also are involved in a number of multicenter grants and industry-sponsored trials. They have also authored scientific statements for national and international societies. UNC Children’s Heart Center faculty collaborate with basic scientists within The University of North Carolina’s School of Medicine Core Laboratories, delving into the molecular mechanisms of cardiovascular disease such as aortic aneurysm formation, genetic malformations of the heart and disease of the heart muscle (cardiomyopathy). It is our hope that one day, these discoveries will move from the bench to bedside to improve the lives of those afflicted with these cardiac conditions. We have recently established a Pediatric Cardiothoracic Surgical Laboratory at UNC. Our surgeons are exploring the impact of flow rates in neonatal and infant piglets with aortic arch hypoplasia regarding somatic (body) perfusion. In addition, collaborating with faculty from NC State School of Biomedical Engineering, we are testing the use of robotic fabric for cavopulmonary assistance in the Fontan circulation of single-ventricle physiology. 21

Humanitarian Work In addition to providing care for all of our state’s citizens, members of the UNC faculty are also involved in providing much needed humanitarian care in parts of the world where such expertise, resources, and infrastructure are lacking. Our UNC cardiologists Drs. Michael Yeung and Elman Frantz, with the help of generous donors from our state, lead trips twice a year to Nicaragua, working in partnership with Project Health for Léon. They perform lifesaving valvular heart repairs in Nicaragua and have established a sustainable presence in that community to provide ongoing training to local providers. Dr. Sharma, who has had a career-long interest and commitment to global surgery, has partnered with Gift of Life International to provide care to children around the world. Recently, he visited Pristina, Kosovo, an independent nation in the previously war-torn Yugoslavian republic with an international team of providers to perform lifesaving surgeries. With the help of Dr. Anthony Charles, director of global surgery at the UNC Institute for Global Health, an effort has been spearheaded to create sustainable pediatric cardiac surgery programs in certain key areas around the world.

UNC Children’s Heart Center Family Advisory Council The UNC Children’s Congenital Heart Family Advisory Council exists to advocate for and support current patients and families in addition to those who have been cared for in the program. Through regular meetings and interactions, this group will address issues of concern and seek to improve the patient experience and outcomes. It affords families the opportunity to play an active role in their children’s care. The Council issues an annual report to the UNC Health CEO (and board of directors) on progress and actions to ensure the Congenital Heart Program is advancing patient- and family-centered care. The UNC Children’s Congenital Heart Family Advisory Council is composed of former and current patients and family members whose children have been treated at UNC Children’s, as well as physicians, clinicians, staff members and local community members. Our membership reflects ethnic, cultural and regional diversity to ensure it is representative of the state of North Carolina.

PATIENT STORY

UNC Health partnerships

JALIAH Ebstein’s Anomaly—The Cone Repair Jaliah was diagnosed prenatally with a rare heart condition called Ebstein’s anomaly occurring in about 1 per 200,000 live births and accounting for <1% of all cases of congenital heart disease. The tricuspid valve that separates the right upper (atrium) and lower (ventricle) chambers of the heart was severely malformed with the valve inserting into the heart in an abnormal position. This usually causes the valve to leak or regurgitate blood backwards into the atrium causing it to stretch. In the most severe form, babies born with this condition might not survive the newborn period. Fortunately, Jaliah had close monitoring for most of her childhood until she developed symptoms including shortness of breath, fatigue with activity, and irregular heartbeats not easily treated with medicine. Recently, Jaliah had her heart valve repaired using the Da Silva “Cone” technique. Additionally, surgeons performed an arrhythmia surgery called the “Maze” procedure, which corrected her irregular heart rhythm so she did not need lifelong medication. Jaliah has recovered fully and is back to playing with her friends. Her care demonstrates the effectiveness of our regional collaboration, allowing children and young adults from around the state to have access to cutting-edge therapies not offered by many heart centers.

across the state of North Carolina allow us to provide a full spectrum of care to our patients. They can receive regional pediatric subspecialty services both in their community as well as quaternary care at the UNC Children’s Hospital in Chapel Hill.

Preoperative image showing apical displacement of tricuspid valve with abnormal attachments into right ventricle

Postoperative image showing “Cone” tricuspid valve repair with restoration of valve to “true” annulus

Outreach It is the mission of UNC

Outreach Partnerships

Children’s to care for

Alamance Regional Medical Center

Johnston UNC Health Care

Burlington, NC

Smithfield and Clayton, NC

Caldwell UNC Health Care

Nash UNC Health Care

Lenoir, NC

Rocky Mount, NC

Clinton Medical Center

Novant Health—New Hanover Regional Medical Center (NHRMC)

children and families across all 100 counties in North Carolina and beyond. Our Congenital

Clinton, NC

Wilmington, NC

Cone Health

Heart Program has

Greensboro and Burlington, NC

defined partnerships to

First Health of the Carolinas

ensure we are serving the state from east to west.

Southern Regional Area Health Education Center Fayetteville, NC

Pinehurst and Rockingham, NC

UNC REX Healthcare

Harnett Health— Betsy Johnson Hospital

Raleigh, NC

Dunn, NC

UNC Hospital Affiliations UNC Rockingham Health Care

UNC REX Healthcare

Caldwell Memorial Hospital Nash UNC Health Care UNC Medical Center Pardee Hospital

Wayne UNC Health Care

UNC School of Medicine Chatham Hospital

Johnston Health

UNC Lenoir Health Care

UNC Health Southeastern Clinton Medical Center Onslow Memorial Hospital Novant Health-New Hanover Regional Medical Center (NHRMC)

Partner With UNC Children’s Heart Center UNC Children’s is committed to improving health in North Carolina and beyond, but we can’t do this without your help. The life-changing work happening every day at UNC Children’s is possible due to families like yours who partner with us in providing the highest quality care possible to our young patients. When you make a gift to UNC Children’s, you share in our commitment to support innovative research and deliver quality, compassionate patient care. Every gift from every donor makes a difference in the lives of our patients, students and faculty. You may designate your gift for a particular purpose, or your gift can be unrestricted, to support the greatest needs and opportunities as they arise. Regardless of where your passion lies, your support will make a significant impact on UNC Health and those we serve. Learn more at unchealthfoundation.org or contact us at supportchildren@med.unc.edu.

Dr. Timothy Hoffman, Albert “Al-J” Jeffries, Dr. Mahesh Sharma and Dr. Scott Buck pose on the field of Kenan Stadium during the UNC Health Kid Champion Program Event. Before the game, just when the anticipation in Kenan Stadium is reaching its peak, the Kid Champion, Al-J, a child who received a heart transplant at UNC Children’s Heart Center, was escorted out to the 50-yard line walking alongside the UNC team captains to attend the coin toss.

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UNC Children’s Heart Center UNC Children’s Hospital 101 Manning Drive Chapel Hill, NC 27514 (984) 974-4601 www.uncchildrens.org 10/21