Tag Archive for: Mary Donofrio

Podcast: Beating strong: The lifelong impact of pediatric cardiology

Supporting healthier hearts

The Children’s National Heart Center is leading the way to find new and better treatments for cardiac problems as early as possible, during pregnancy and just after birth.

These critical stages are vital for a child’s lifelong heart health. “The innovations we are pursuing have the potential to transform the landscape of cardiac treatment,” says Wayne Franklin, M.D., F.A.C.C., Heart Center senior vice president.

“By focusing on the earliest stages of life, we can significantly alter the trajectory of children’s heart health, creating a lifetime of possibilities.”

Mending a Broken Heart

Abby with her sister and parents

Abby with her sister and parents.

Abby, who just turned 1, is a smiley baby who loves to play peek-a-boo with her sister, Ruby. “We marvel that she is perfectly healthy,” says her father, Dan. He and wife Kelsey love to linger over ordinary moments. Her given name, Abigail, means “a father’s joy.”

When Kelsey was 18 weeks pregnant, she and Dan learned their baby had signs of heart injury, which led to a dangerous rhythm problem called “complete heart block.” The previous year, their infant son died from the same condition, which was discovered too late. The family prepared for another loss. But early detection of the problem and advanced care that started in the womb made all the difference for Abby. Children’s National prenatal cardiology experts began monitoring Abby’s development from the earliest possible moment and were able to intervene before devastating injury occurred.

Before Abby’s diagnosis, in light of the previous pregnancy, Kelsey enrolled in a clinical trial. The research sought a better way to identify and treat the heart condition Abby was at risk for. Anita Krishnan, M.D., a pediatric cardiologist and clinician scientist, met the family during their initial visit and arranged a monitoring plan that included frequent visits to make sure Abby’s heart was working normally. Soon after Kelsey’s first visit, doctors noted a problem.

Mary Donofrio, M.D., F.A.A.P., F.A.C.C., F.A.S.E., a leading pediatric and fetal cardiologist and The Van Metre Companies Professor of Fetal Cardiology, led the team that initiated lifesaving in utero therapy, followed Abby’s progress in the womb and planned for her arrival. The goal was to extend the pregnancy for as long as possible so she would survive birth and the heart surgery that would follow.

Abby’s prognosis improved as weeks passed. As a newborn, she would be a candidate for an infant pacemaker the size of a penny. It would help regulate her heartbeat and enable her to live a “normal” life.

Pericardial port for pediatric pacemaker delivery

Pericardial port for pediatric pacemaker delivery, developed by Dr. Charles Berul and team.

Kelsey and Dan met with Charles Berul, M.D., emeritus chief of Cardiology and The Van Metre Companies Professor of Cardiology, and his team. There was uncertainty about whether Abby’s heart was too damaged for the pacemaker to work, but Dr. Berul, who has spent decades refining designs for this type of device, expressed confidence. Abby would be the world’s 27th infant, and the fifth at Children’s National, to have one implanted.

“To have him say, ‘We’ve developed this device, we know what we’re doing, all of the other babies who have had this are doing well and we’ll be right here in the room with you,’ was pretty incredible,” Dan says.

Kelsey’s monitoring in the clinical trial and the innovative therapy that started before birth likely helped Abby survive until she was born at 32 weeks at MedStar Washington Hospital Center. Dr. Donofrio and the Children’s National care team were in the delivery room and rushed Abby to our Cardiac Intensive Care Unit. Dr. Donofrio arranged for mom and daughter to pass in the hall on the way. “I heard her cry and felt relief for the first time,” Kelsey says.

Abby’s pacemaker enabled her heart to beat properly on its own. She soon moved to our Neonatal Intensive Care Unit. In two months, she went home with her family. Kelsey and Dan monitor her pacemaker with a handheld device that sends reports to her team at the hospital.

“We’re lucky to have doctors nearby who are at the forefront of this lifesaving research. Children’s National took care of us with a great deal of humanity. Now we can focus on being a family,” says Dan.

Read more stories like this one in the latest issue of Believe magazine.

Fetal detection, risk stratified care algorithms give infants with CHD their best chance to thrive

Mary Donofrio, M.D., medical director of Prenatal Cardiology at Children’s National Hospital

Mary Donofrio, MD, medical director of Prenatal Cardiology at Children’s National, and other dedicated pediatric cardiologists working in this evolving specialty have spent most of the last two decades defining the field.

Congenital heart disease (CHD) can be detected in utero with precision and accuracy. With advanced technology, identification of a problem happens earlier than ever, including identifying details that predict whether a baby may be dangerously sick at birth. This gives fetal and pediatric cardiologists time to make plans for delivery and specialized care immediately after birth. These critical first moments can be the key to survival for infants with the most complicated defects.

Mary Donofrio, MD, medical director of Prenatal Cardiology at Children’s National Hospital, and other dedicated pediatric cardiologists working in this evolving specialty have spent most of the last two decades defining the field and demonstrating the importance of making sure every child with a congenital heart defect is diagnosed as early as possible to give them the best chance for a healthy life.

Children’s National leads the way

Children’s National performs more than 4,000 fetal ultrasounds each year to detect and manage the unborn child with congenital heart disease, making it one of the most experienced centers in the United States at finding these conditions and planning for their care.

For more than 20 years, every fetus diagnosed with congenital heart disease at Children’s National following an obstetrician referral has their anticipated level of delivery room care assigned by a fetal cardiologist. Protocols were created at Children’s National and validated to establish specialized delivery room management for each patient. The management plan includes specifics about the time and place of delivery and which delivery room staff members are required for stabilization and care after birth based on the severity of the condition.

The outcomes from this approach were published in a landmark 2013 study showing the impact on improving outcomes for infants with the most serious forms of congenital heart disease. Since then, these protocols have become part of more extensive fetal cardiology care guidelines that are in use both at Children’s National and around the world.

“The guidelines we wrote include recommendations about who should get a fetal echo, how to do a fetal echo, how to manage babies in utero including when a fetal intervention might be necessary, and finally how to decide the level of cardiology care that should be present in the delivery room,” according to Donofrio, who served as lead author.

In Washington, D.C., approximately 60 to 75% of congenital heart defects are diagnosed before a baby is born, giving doctors and other care providers critical days, weeks and months to plan how best to protect the fragile infant during their transition into the world from the safe haven of their mother’s body.

What’s next

Fetal imaging guidelines tell obstetricians which expecting mothers should be referred for a fetal ultrasound given a higher level of risk for CHD over the population risk. However, most women do not have any risk factors that will trigger additional testing beyond obstetrical screening. Also, many families even if referred are far from a center that is qualified to perform a fetal echocardiogram to detect these conditions.

Research at Children’s National, led by Anita Krishan, MD, and Dr. Donofrio in collaboration with the Fetal Heart Society, an international research collaborative, showed that in the U.S., factors such as socio-economic status, ethnicity and geography are important barriers to detection of severe congenital heart diseases such as hypoplastic left heart syndrome and transposition of the great arteries.

In a follow-up study by Jennifer Klein, MD, and Dr. Krishnan, distance was not the only barrier to detecting CHD, however. Geo-mapping technology using zip codes allowed the team at Children’s National to pinpoint “hot spots” where detection is decreased, even in places where care should be available. The Heart Center team is hoping to work with providers in these neighborhoods to improve access to care and help educate local clinic providers about how to image and when to refer for further testing.

Donofrio and colleagues are also working to develop ways to improve the diagnosis of fetal heart disease in places that are far from the Heart Center. This includes exploring more portable diagnostic tools and applying telehealth strategies to connect fetal heart experts with local care providers to make an action plan, before a baby arrives potentially in distress. In addition, a phone-based application is under development to help sonographers to identify abnormal images in real time during routine scans in remote locations. Improved detection rates have also opened the doors to powerful new studies investigating how maternal health and stress impacts brain development in fetuses with congenital heart disease. Ongoing research looks at ways to better support expecting mothers, with the goal of helping moms cope with stress during pregnancy so her baby has the best chance possible to be born healthy and strong.

Donofrio says she won’t stop until in utero detection of congenital heart disease is 100%. “Where you live, your neighborhood, your life experience or how far you live from the Heart Center, should not decrease our ability to do everything possible to care for every baby and achieve the best outcome possible,” she says.

Tracking neurodevelopmental outcomes for kids with congenital heart disease

Extensive research has shown that children with congenital heart disease (CHD) who are born blue or who need cardiac surgery in their first year of life are at risk for developmental challenges and/or learning difficulties.

Mary Donofrio, M.D., co-director of the Cardiac Neurodevelopment Outcome (CANDO) program at Children’s National Hospital, says that we started the program — the only one of its kind in the Washington, D.C. region — to identify and manage delays in development and difficulties with learning, no matter when they arise.

“We start paying attention even before birth and then continue to evaluate neurodevelopment at key stages in a kid’s life to assure the best outcome. Our goal is for every kid born with CHD to be able to achieve their full potential, be active, make friends and succeed in school. Most important, we want each of our patients to grow up to be a happy and successful adult,” says Dr. Donofrio.

Learn more about CANDO at Children’s National Hospital and our role in developing best practices for neurodevelopmental and psychosocial services as part of the international multi-specialty Cardiac Neurodevelopmental Outcome Collaborative.

Mary Donofrio, M.D., FAAP, FACC, FASE, named as The Van Metre Companies Professor of Fetal Cardiology

Mary Donofrio

Children’s National Hospital named Mary Donofrio, M.D., FAAP, FACC, FASE, as The Van Metre Companies Professor of Fetal Cardiology at Children’s National Hospital.

Children’s National Hospital named Mary Donofrio, M.D., FAAP, FACC, FASE, as The Van Metre Companies Professor of Fetal Cardiology at Children’s National Hospital.

Dr. Donofrio serves as Medical Director of the Prenatal Cardiology Program and Critical Care Delivery Program, Director of the Advanced Cardiac Imaging Fellowship and Co-Director of the Cardiac Neurodevelopmental Outcome Program at Children’s National. She is a Professor of Pediatrics at George Washington University and is the founding and current President of the Fetal Heart Society, a non-profit organization created to advance the field of fetal cardiovascular care and science through collaborative research, education and mentorship.

About the award

Dr. Donofrio joins a distinguished group of 42 Children’s National physicians and scientists who hold an endowed chair. Professorships at Children’s National support groundbreaking work on behalf of children and their families and foster new discoveries and innovations in pediatric medicine. These appointments carry prestige and honor that reflect the recipient’s achievements and donor’s forethought to advance and sustain knowledge.

Dr. Donofrio is an international expert in fetal cardiology. She specializes in the fetal diagnosis and assessment of cardiovascular disease and the in-utero and delivery room management of newborns with complex congenital heart disease (CHD). Dr. Donofrio created an evidenced-based risk-assessment protocol for delivery room management which is now the standard of care for newborns with CHD. Dr. Donofrio has been a co-investigator on several NIH sponsored studies assessing in utero factors that influence neurodevelopmental outcome in children with CHD and more recently a study designed to minimize brain injury during heart surgery using cardiopulmonary bypass. She has published more than 130 papers, including the American Heart Association Scientific Statement on the Diagnosis and Treatment of Fetal Cardiac Disease.

The Van Metre Companies, through their vision and generosity, are ensuring that Dr. Donofrio and future holders of this professorship will launch bold, new initiatives to rapidly advance the field of fetal cardiology, elevate our leadership and improve the lifetimes of children with special hearts.

About the donors

For the past 65 years, Van Metre Companies has remained one of the Greater Washington D.C. area’s most successful, private, multi-faceted real estate developers. Albert G. Van Metre, the founder of Van Metre Companies, established a tradition of philanthropy focused on local charities. As a homegrown business, perpetuating that legacy of local giving is both a responsibility and a source of pride. The Van Metre Companies Professor of Fetal Cardiology honors Albert G. Van Metre’s memory by continuing this tradition of commitment to the community they call home.

The Van Metre Companies hosts the Annual Van Metre 5K Run in support of Children’s National, a longstanding tradition that has raised nearly $3 million in the past 30 years. In 2010, Children’s National dedicated the Van Metre Companies Cardiovascular Surgery Operating Room, a state-of-the-art cardiovascular surgery suite which was funded through the Annual Van Metre 5K Run. They also established The Van Metre Companies Professorship in Cardiology held by Charles Berul, M.D., Chief of Cardiology and Co-director of Children’s National Heart Institute.

 

R01 grant funds white matter protection study for congenital heart disease

Nobuyuki Ishibashi

Nobuyuki Ishibashi, M.D., is the principal investigator on a $3.2 million NIH R01 to study white matter growth and repair in utero for fetal brains affected by congenital heart disease.

Many of the neurological deficits seen in children with congenital heart disease (CHD) are related to abnormal white matter development early in life caused by reduced oxygen supply to the brain while in utero. Children with immature white matter at birth also commonly sustain additional white matter injuries following cardiac surgery.

The NIH recently awarded a prestigious R01 grant totaling more than $3.2 million to a collaborative project led by the Center for Neuroscience Research, the Sheikh Zayed Institute for Pediatric Surgical Innovation and the Children’s National Heart Institute at Children’s National Hospital as well as MedStar Washington Hospital Center.

The research, titled “White matter protection in the fetus with congenital heart disease,” looks specifically at whether providing a supplemental amount of the naturally occurring tetrahydrobiopterin (BH4) for pregnant women could rescue white matter development of fetuses with congenital heart disease whose brains aren’t receiving enough oxygen – or suffering from hypoxic-ischemic events.

Previous preclinical studies have shown that this lack of oxygen depletes the brain’s natural BH4 level, and the researchers hypothesize that BH4 levels play a critical role in the growth and development of white matter in the fetal brain by triggering key cellular/molecular processes. Specifically, the study will focus on three aims:

  1. Establish in a preclinical model the optimal protective regiment for women pregnant with a fetus who has CHD to receive BH4.
  2. Determine the appropriate approach to deliver BH4 to this population
  3. Leverage genetic tools and biochemical techniques in the laboratory to better understand where and how BH4 levels play a role in the growth (or lack thereof) of oligodendrocytes—the primary cells of white matter.

This laboratory-based work is the first step to determining if the neurodevelopment of babies born with CHD can be preserved or recovered by addressing key brain development that occurs before the baby is even born. Findings related to congenital heart disease may also translate to other populations where white matter development is affected by hypoxia-ischemia, including premature infants.

The project is led by principal investigator Nobuyuki Ishibashi, M.D., with co-investigators Vittorio Gallo, Ph.D., Joseph Scafidi, D.O., and Mary Donofrio, M.D. as well as colleagues at MedStar Washington Hospital Center.