Tag Archive for: pediatric cardiology

The multi-disciplinary team who implanted the first AVEIR leadless pacemakers at Children’s National.

Leadless pacemakers, subcutaneous defibrillators successfully implanted in pediatric patients

The multi-disciplinary team who implanted the first AVEIR leadless pacemakers at Children’s National.

The multi-disciplinary team who implanted the first AVEIR leadless pacemakers at Children’s National.

Two new devices being used in adults with heart rhythm disorders — atrial and dual chamber leadless pacemakers and extravascular defibrillators — were successfully implanted in pediatric patients for the first time at Children’s National. These devices represent the latest technology in pacing and defibrillating the heart to maintain its rhythm. Though they are smaller in size, have fewer complications and longer battery life than most of the devices currently available for young patients, they have not been available for use in these younger patients until recently.

“For the first time, we’re bringing these devices that are smaller, smarter, less painful and more flexible to children and teenagers who can really benefit from them,” says Elizabeth Sherwin, MD, a pediatric cardiologist and electrophysiologist at Children’s National who led the teams completing these minimally invasive procedures.

The patient benefit

Offering implantation of these devices gives more children and adults with congenital heart disease access to the latest technologies in implantable heart rhythm devices, which may offer unique benefits for these groups.

Abbott AVEIR dual chamber leadless pacemaker is the newest FDA approved leadless pacemaker. It uses electricity to maintain heart rhythm and can be used to pace both the top and bottom chambers of the heart, which is particularly important for pediatric and adult congenital patients. These devices also:

  • Are designed to be removed and replaced after battery runs down, which is ideal for children and young adults who will have multiple replacements over a lifetime.
  • Long battery life, so fewer replacements may be necessary.
  • Can be placed minimally invasively

Dr. Sherwin says that the minimally invasive procedure and the lack of leads on these devices are particularly key for younger people because these factors remove or reduce the risk of complications commonly experienced with pacemakers in children. There is a reduced risk of bleeding, infection, lead movement or fracture, and long-term problems with the veins.  Even better, because they are placed directly in the heart, there are no scars on the chest or visible signs of a pacemaker present.

Medtronic Aurora EV-ICD is an extravascular implantable cardioverter-defibrillator (ICD), which is implanted under the skin (subcutaneous) with a generator on the left chest wall and a lead that goes under the breastbone (sternum). The design includes:

  • A smaller generator.
  • No need to go through chest muscle, leading to less painful implantation and more comfort long term.
  • Emergency heart pacing through the substernal lead – both to try to terminate a fast arrhythmia to avoid need for a shock, and to treat in case the heartbeat is too slow.
  • Longer battery life (projected 11 years).
  • Avoids the need to have leads in the blood vessels, with the many potential complications that go along with transvenous leads.

For both of these newer devices, the patient’s size, weight and medical history will help determine if they are a good candidate.

The big picture

The Electrophysiology team is the first and only pediatric and congenital cardiology team trained to implant AVEIR leadless pacemakers  and the Aurora EV-ICD for eligible individuals in the mid-Atlantic region. Dr. Sherwin, Charles Berul, MD, and Tom (Nak) Choi, MD, are trained to provide these procedures for people in Washington, DC, Virginia and Maryland. For both devices, Children’s National is among only a handful of children’s hospitals in the U.S. with the training and expertise to offer access to these technologies.

“This is a game-changer for kids with rhythm disorders and adults with congenital heart disease,” Dr. Sherwin says. “We are really excited to be among the first to offer these options for patients who need them.”

The Children's National Heart Center team

Children’s National co-leads efforts to increase skin-to-skin care for babies with congenital heart disease

The Children's National Heart Center team

The Children’s National Heart Center team led activities designed to encourage skin-to-skin contact between parents and infants in the Cardiac Intensive Care Unit and Heart and Kidney Unit.

Clinicians at Children’s National Hospital and Children’s Hospital Orange County are leading a nationwide event to encourage families to practice more skin-to-skin, or kangaroo, care with newborn infants who have congenital heart disease, including throughout hospitalization.

Thirty-one hospitals across the United States will participate in this congenital heart disease focused “Skin-to-skin-a-thon,” that will include family and clinical care provider activities and education throughout pediatric cardiac intensive care units and step-down units.

The event will celebrate the tremendous benefits that research shows both families and infants gain from physical contact early in life.

Early skin-to-skin care has been shown to:

  • Reduce stress in both baby and the parent
  • Help with baby’s physiologic stability including regulating vital signs like temperature, heart rate, and blood pressure
  • Provide infant pain relief
  • Improve infant digestion and weight gain
  • Support good sleep/wake cycles in babies.
  • Increase oxytocin for mothers, which can help improve milk production/support breastfeeding

Most studies showing these benefits have included pre-term babies or those born after a healthy term. The idea of encouraging family skin-to-skin care in the hospital setting has been widely adopted in neonatal intensive care units but is not done routinely in cardiac intensive care units. One study estimated that only 6% of parents whose babies were hospitalized for congenital heart disease reported any skin-to-skin care during their stay, with most stays averaging 22 days.

“Research shows so many benefits for all infants and their parents — and our congenital heart newborns stand to gain even more from this type of contact, but often receive it far less,” says Sarah Schlatterer, MD, PhD, medical director of Neurocardiac Critical Care at Children’s National. “This awareness effort is designed to help families understand how to do this safely and also empower our bedside care providers to encourage skin-to-skin care as much as they can every day.”

The event overall is inspired and supported by the Cardiac Newborn Neurodevelopmental Network SIG of the Cardiac Neurodevelopmental Outcomes Collaborative, who planted the seed of the idea and assisted with dissemination of information and coordinating between participating hospitals.

3D model for heart valve replacement planning

Living tissue heart valve replaces mechanical mitral valve through partial heart transplant

3D model for heart valve replacement planning

3D model for heart valve replacement planning.

An 11-year-old boy is the first in the world to have an artificial heart valve replaced with a live tissue valve from a donated heart through a partial heart transplant. The procedure took place at Children’s National Hospital in Washington, D.C. The successful surgery, performed by the cardiac surgery team, is also the region’s first partial heart transplant.

“I am honored this family trusted our hospital and our team’s expertise to perform this life-changing first-of-its-kind procedure for Preston,” says Cardiac Surgery Chief Yves d’Udekem, MD, PhD. “I look forward to hearing about all the new activities and adventures he and his family can do once he is completely recovered from surgery.”

Heart valve recipient Preston

“Everyone is ecstatic with his progress so far,” says Lauren Porter, who is the patient’s mother. “We hope having this surgery will give him a lot more freedom to do the things he loves in his life, and we hope that by sharing our story we are helping to make procedures like this more available to kids who need them in the future.”

Artificial heart valves are the standard of care for a failing valve in a child born with congenital heart disease. But Dr. d’Udekem says they are exceptionally difficult in children. First, a traditional artificial tissue valve lasts only about a decade, so children like Preston who have their first valve inserted before age 2 will inevitably face at least two to three additional open-heart surgeries before age 40. Additionally, just like adults, an artificial mechanical heart valve requires the patient to take blood thinners and major precautions against injury for their entire lives. Research has also shown that the placement of an artificial heart valve causes the heart to change shape over time, impacting heart function later in life and leading to a shortened life span.

Replacing this valve with a living transplanted valve will give Preston freedom from a lifetime of blood-thinning medication. Research also shows these live tissue implants should grow along with him, greatly decreasing the likelihood of future open-heart surgeries.

“Everyone is ecstatic with his progress so far,” says Lauren Porter, who is the patient’s mother. “We hope having this surgery will give him a lot more freedom to do the things he loves in his life, and we hope that by sharing our story we are helping to make procedures like this more available to kids who need them in the future.”

Children’s National is the first hospital to remove a child’s previously implanted artificial valve and replace it with a live working valve from a donated heart through a relatively new procedure called a partial heart transplant. The Children’s National partial heart transplant replaced the heart’s mitral valve, which is the valve between the left upper chamber (left atrium) and the left lower chamber (left ventricle) of the heart. In general, partial heart transplants are rare. Prior to this surgery, four U.S. hospitals have used partial heart transplants to replace a failing, living heart valve with a valve from a donor heart, but no organization to date has ever replaced a prosthetic valve with a real one.

“Making this procedure an option for certain children who need a heart valve replacement is critical to having patients live their best lives and to providing hope to their family as they grow into adolescence and adulthood,” says Wayne J. Franklin, MD, senior vice president of the Children’s National Heart Center and a congenital cardiology specialist. “I am proud of our team that conducts such important research to innovate better clinical solutions for all of our patients with congenital heart disease.”

Live tissue partial heart transplants also offer an additional benefit. Donated hearts that do not qualify for use in a total heart transplant may have healthy components, like valves, that can be used for patients who don’t require total replacement. Candidates and potential donors are listed in a registry and matched according to biological factors including blood type, similar to the process for determining full heart transplant candidates.

Dr. Wayne Franklin and patient

Qualities of successful pediatric and congenital cardiovascular programs: Cardiology 2025

Dr. Wayne Franklin and patient

Dr. Franklin’s talk offered his observations of how the administrative backbone behind clinical care supports a thriving center for infants and children with congenital heart disease and their families.

Wayne Franklin, MD, FACC, senior vice president of Children’s National Heart Center, joined a panel discussion at Cardiology 2025: The 28th Annual Update on Pediatric and Congenital Heart Disease. The panel, Healthcare Administration in Pediatric and Congenital Cardiovascular Disease: Sharing Challenges and Creating Solutions, sought to identify the traits that successful U.S. healthcare programs, and especially pediatric cardiovascular programs, have in common.

Dr. Franklin’s talk, “Structuring Administration for Pediatric & Congenital Cardiovascular Care,” offered his observations of how the administrative backbone behind clinical care supports a thriving center for infants and children with congenital heart disease and their families.

The big picture

Dr. Franklin noted that the best programs are finding successful combinations of the right ingredients to make the “secret sauce” — focus on high quality care being delivered in ways that are financially sustainable. More than ever, this is hard to accomplish.

Today’s pediatric programs are often organized in an academic model where clinicians are employed by a university or medical school, typically within a Department of Pediatrics, with pediatric subspecialties all falling together under that department. While the academic model has been successful up to this point, there may be valuable lessons to learn and opportunities for further success by looking at outcomes from other models in the broader adult healthcare sector.

Key takeaways

Dr. Franklin offered several examples of key models for pediatric heart centers to consider. They include:

  • A multi-disciplinary, “service-line centered” structure: All doctors, nurses, advanced practice providers and support staff aligned together under a “center” or “institute” model, similar to the Cleveland Clinic’s Institutes of Excellence.
  • A blended, “privademic” structure for clinicians, where they are direct employees of a hospital, but not of a larger university or healthcare system.
  • Clinician leadership and engagement in business administration structure and function, to make sure that patients remain front and center in business decisions.
  • Dedicated quality and safety teams that are driven by data and outcomes, foster frequent and early communication and ensure care providers actively engage with these efforts.
  • A model that supports innovations in care and investments in research to continue advancing best practices for patients and families.
  • A system for education and training to make sure the next generation can effectively carry on the established culture of excellence.

What matters most

No matter the structure, the most important and common theme among successful health systems, hospitals and even specific heart programs, is steadfast, organization-wide dedication to decision making driven by what is best for patients. This approach should drive a focus on early detection and/or prevention, and lead to positive outcomes, which ultimately brings financial sustainability.

See more about Children’s National at this year’s meeting: Cardiology 2025: 28th Annual Update on Pediatric and Congenital Cardiovascular Disease.

Doctors performing cardiac surgery at Children's National Hospital

Socioeconomic disadvantage associated with higher long-term mortality after heart surgery

Doctors performing cardiac surgery at Children's National Hospital

Children who had heart surgery and come from less advantaged neighborhoods in the Washington, D.C., region are much more likely to die in the long term than those from neighborhoods with more wealth and opportunity.

Children who had heart surgery and come from less advantaged neighborhoods in the Washington, D.C., region are much more likely to die in the long term than those from neighborhoods with more wealth and opportunity. The finding was part of a presentation, Socioeconomic Disadvantage Is Associated with Higher Long-Term Mortality After Cardiac Surgery, presented by Jennifer Klein, MD, MPH, cardiologist at Children’s National Hospital, during the Society of Thoracic Surgeons (STS) Annual Meeting in Los Angeles.

What is it?

Clinical and demographic data were collected for 2,546 people who underwent corrective surgery for congenital heart disease over a 15-year period (2007-2022) in a single-center retrospective analysis. Using the Child Opportunity Index (COI), an index that uses environmental/health, educational, and socioeconomic domains to assess patients’ neighborhoods, the two lowest COI quintile neighborhoods were designated as “disadvantaged” while the two highest COI quintile neighborhoods were designated as “advantaged.” Multivariable mixed model analyses were conducted to understand the factors associated with overall mortality.

The research showed that patients from disadvantaged neighborhoods suffered in a great proportion both overall mortality [n=168 (13.6%) vs 66 (8.4%), p< 0.001] and major complications [n=168 (13.6%) vs 81 (10.3%), p< 0.001]. The positive association between neighborhood disadvantage and long-term mortality was maintained in stepwise logistic regression multivariable analysis after controlling for differences between surgical complexity and other clinical risk factors.

What it means

“Previous research has shown disparities like this,” says Dr. Klein. “What’s significant here is that the disparity for children from disadvantaged neighborhoods grows over time. That means corrective interventions won’t be implemented in the operating room or within the first 30 days after surgery. They need to happen in a cardiologist’s office, in the schools, and in the community long after a child goes home from the hospital.”

The findings from this particular study are also noteworthy as they are the first to include significantly greater numbers of patients who are more likely to experience this risk (48.5% of the study group). Previous research has sought to look at this research question but analysis may have been skewed by an over-representation of socioeconomically advantaged patients.

What’s next

Differences in mortality risk continue to grow greater as more time passes, the authors note, indicating that the disparity is not a product of surgical technique or disparities in care delivery at the time of surgery.

Instead, their outcomes are being influenced by factors outside the operating room. This opens a new avenue of research focused on identifying the “why.”

“What happens after they go home?” Klein asks. “Can they access medications? Do they come back for follow-up appointments? Can they afford food?”

Read the abstract, Socioceconomic Disadvantage Is Associated with Higher Long-Term Mortality After Cardiac Surgery, which was presented during a speed session at the STS Annual Meeting. A full manuscript will be published soon.

American Heart Association Scientific Sessions logo

Children’s National Hospital at American Heart Association Scientific Sessions 2024

American Heart Association Scientific Sessions logoExperts from Children’s National Heart Center presented and shared their latest research findings at this year’s American Heart Association (AHA) Scientific Sessions, held in Chicago, Illinois, in mid-November.

The annual AHA Scientific Sessions are attended by scientists, clinicians, researchers and other health care professionals from around the globe who have an interest in cardiovascular disease. Children’s National Hospital experts highlighted work focused on caring for the full spectrum of people who live with congenital heart disease — from tiny neonates through adulthood.

Presentations

  • Transvenous cardiac re-synchronization: When is it effective in CHD? Charles Berul, M.D.
  • Leveling the Playing Field: Creating Equity within Pediatric Cardiology Leadership and Salary, Wayne Franklin, M.D.
  • Debate: Patients with small coronary artery aneurysms SHOULD be discharged – US experience, Ashraf Harahsheh, M.D.
  • Science of Engagement: Inclusion of Adults with Congenital Heart Disease Living with Neurodevelopmental Disability in PCOR, Anitha John, M.D., Ph.D.
  • 100 Years of AHA Leading Global Health, Craig Sable, M.D.
  • Best Oral Abstract: Safety of Discontinuing Secondary Antibiotic Prophylaxis After Echocardiographic Normalization in Early Rheumatic Heart Disease, GOAL-Post Study, Craig Sable, M.D., co-author
  • Su2032│CMR can discriminate need for biopsy and rejection therapy in children post heart transplant, Ravi Vamsee Vegulla, M.D.

Posters and poster presentations

  • Minimally-Invasive Intrapericardial Injections under Direct Visualization via Thoracic Cavity Access in Infant and Pediatric-sized Pre-clinical Model, Charles Berul, M.D., Ryan O’Hara, Ph.D.
  • Early total cfDNA, but not donor fraction, predicts late events after heart transplantation, Shriprasad Deshpande, M.D.
  • Impact of Angiotensin Receptor Neprilysin Inhibitor on Chronic Heart Failure with Reduced Ejection, Shriprasad Deshpande, M.D.
  • Fraction in Adult Congenital Heart Disease Patients: A Systematic Review and Meta-analysis, Shriprasad Deshpande, M.D.
  • Trough Level Prediction of Major Adverse Transplant Events: A Report from the TEAMMATE Trial, Shriprasad Deshpande, M.D.
  • Thrombocytosis is Prevalent and Associated with Greater Inflammation and Coronary Artery Involvement in Both Kawasaki Disease and Multisystem Inflammatory Syndrome in Children Associated with COVID-19, Ashraf Harahsheh, M.D.
  • Mesenchymal Stromal Cell Delivery through Cardiopulmonary Bypass in Pediatric Cardiac Surgery – MeDCaP Phase I Trial, Nobuyuki Ishibashi, M.D., Shriprasad Deshpande, M.D., et. al.
  • Social Determinants of Health: Impact on Mortality and Care Status for Adults with CHD, Jamie Jackson, Ph.D.; Anitha John, M.D., Ph.D., co-author
  • Loss to Follow-Up Among Adults with Congenital Heart Defects: A Report from Congenital Heart Disease Project to Understand Lifelong Survivor Experience (CHD PULSE), Anitha John, M.D., Ph.D., co-author
  • The Burden of Adult Congenital Heart Disease in the United States, Vasupradha Suresh Kumar, M.D.
  • Determining the Physiologic Effect of the Cavopulmonary Connection on Caval Flows Using 4D Flow MRI , Vasupradha Suresh Kumar, M.D.
  • Shape Variations in Right Ventricular 3D Geometry are associated with adverse outcomes in Hypoplastic Left Heart Syndrome Patients: A Fontan Outcomes Registry using CMR Examination (FORCE) Study, Yue-Hin Loke, M.D.
  • Matrix Metalloproteinases and Tissue Inhibitors of Metalloproteinases as Biomarkers in Duchenne Muscular Dystrophy Cardiomyopathy, Christopher Spurney, M.D., co-author
  • Duchenne Muscular Dystrophy Boys Have Diastolic Dysfunction Based on Cardiac Magnetic Resonance, Christopher Spurney, M.D., co-author

Read more about these presentations and posters on the AHA’s meeting website: Programming – Scientific Sessions 2024.

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

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.

Pulse oximiter on baby's foot

AAP updates newborn screening recommendations used to detect critical CHD

The American Academy of Pediatrics (AAP) has updated their clinical recommendations for a crucial heart screening protocol for newborn infants. The simple, noninvasive screening using pulse oximetry has been part of the U.S. Recommended Uniform Screening Panel since 2011. Today, it is a required part of newborn screening in all 50 states.

A clinical report containing the updates was published in the Pediatrics January 2025 edition (online December 16, 2024). The report, “Newborn Screening for Critical Congenital Heart Disease: A New Algorithm and Other Updated Recommendations,” includes endorsement of several new updates for health care providers as well as some other key takeaways from the first 14 years of national screening implementation.

Protocol updates reduce time from screening to intervention

The AAP recommendations refine the already successful protocol to ensure the screening is applied consistently, accurately and efficiently. Changes include:

  • A more simplified CCHD screening algorithm that eliminates a second verification re-screen so treatments can start sooner when intervention is urgent.
  • Oxygen saturation measurements of 95% or greater in both the right hand and either foot for an infant to pass. Previously 95% or greater oxygen saturation measurements in the right hand or either foot would pass.

A lifesaving unintentional benefit

The report also highlights an important, unintended benefit of pulse-oximetry screening that became evident after long-term implementation: The screening protocol also detects critical noncardiac conditions such as sepsis and pneumonia that benefit from early identification and treatment in vulnerable newborns. In fact, the authors note that for every case of critical congenital heart disease detected, four or five cases of infections or respiratory causes of low oxygen saturation are identified.

Recommendations for the next decade

The clinical report authors also note several recommendations for continued implementation of the screening algorithm.

  • Screening with pulse oximetry is best when paired with fetal ultrasounds and newborn examination. It should not be used alone to determine whether an infant has critical congenital heart disease.
  • Data collection, data sharing and improved access to care, including electronic data exchanges and stronger collaborations between birth hospitals and public health programs, are critical for these screening protocols to serve as tools to improve outcomes for children born with congenital heart disease.

“The earlier we can detect these conditions, the earlier we can treat these babies during their first days of life,” said Gerard R. Martin, M.D., M.A.C.C., F.A.H.A., F.A.A.P., senior author and pediatric cardiologist at Children’s National Hospital. “The timely coordination of care saves lives and has proven to be cost-efficient. The routine and uniform use of screening at every medical center is essential to ensure equity. We want all newborns born with critical congenital heart disease to benefit from screening no matter where they were born.”

Children’s National leads the way

Dr. Martin and colleagues across the country continue work to research and refine the algorithm with a focus on standardizing the application for more uniform results and raising awareness of the need for continued collaboration and cross-institutional data sharing to improve outcomes nationwide.

Since 2009, Dr. Martin and nurses at Children’s National have been part of the national cohort of clinicians who advocate for this screening for newborns in every birthing hospital. Findings from long-term implementation studies conducted by Children’s National and Holy Cross Health in Maryland have helped further refine the algorithm and establish the scientific evidence for its benefits and effectiveness.

Read the AAP’s press release about the updates: AAP updates recommendations on use of pulse oximetry to screen newborns for critical congenital heart disease.

winners of the pediatric medical device competition

Winners announced in pediatric medical device competition focused on cardiology

winners of the pediatric medical device competitionSix medical technology innovators focused on pediatric cardiology were selected to receive grants of $50,000 each in the “Make Your Medical Device Pitch for Kids!TM” competition in Toronto. The funds will help awardees bring their devices to the market and improve care for children with heart conditions.

The awardees, selected from a highly competitive field of ten finalists, are:

  • Bloom Standard, Minneapolis – Autonomous, hands-free ultrasound
  • Compremium AG, Bern, Switzerland – Noninvasive central venous pressure estimation for pediatric patients
  • Massachusetts Institute of Technology, Cambridge, Mass. – Polymeric auxetic stent to treat pediatric aortic coarctation
  • OxiWear, Arlington, Va. – Home measurement of oxygen levels in pediatric congenital heart disease
  • PyrAmes Inc., Cupertino, Calif. – Improved, wearable, noninvasive pediatric blood pressure monitor
  • Sibel Health, Chicago – Hospital-to-home monitoring for pediatric heart conditions

The competition is presented by the Alliance for Pediatric Device Innovation (APDI), a nonprofit consortium led by Children’s National Hospital and funded through the Food and Drug Administration (FDA), and Additional Ventures, a nonprofit focused on accelerating research progress and improving clinical care for individuals born with single ventricle heart defects. Along with grant funding, awardees gain access to support services and technical expertise provided by APDI and Additional Ventures in areas that include engineering, regulatory, reimbursement, clinical trials study design and data science services.

According to the Centers for Disease Control and Prevention, about 40,000 children are born annually with a congenital heart defect. Children with heart conditions need medical devices tailored to their specific physiological needs. There is a significant unmet need for pediatric devices designed to monitor and treat young patients effectively in cardiology, interventional cardiology, cardiac surgery and electrophysiology. This competitive grant program is designed to identify and support the development and commercialization of devices addressing these needs.

“Congratulations to our awardees, whose innovative technologies show great promise in advancing care for pediatric heart patients,” said Kolaleh Eskandanian, Ph.D., M.B.A., vice president and chief innovation officer at Children’s National and APDI program director and principal investigator. “We are thrilled to welcome this new cohort into our pediatric device accelerator, where they will have the opportunity to collaborate with clinician-scientists at Children’s National and connect to Additional Ventures’ network. Along with these collaborations, the awardees will benefit from a full range of APDI wraparound services designed to support the development of devices specifically for pediatric patients, helping them navigate the complex path to market.”

The competition was held in conjunction with the 12th Annual Symposium on Pediatric Device Innovation, presented by Children’s National and co-located with The MedTech Conference powered by AdvaMed.  Focused on transforming pediatric care with exclusive innovations for children, this year’s symposium featured panel discussions and keynote presentations with leading experts in pediatrics and medical technology to exchange information and ideas on critical issues in pediatric device development and pediatric healthcare innovation gaps.

“Additional Ventures is thrilled to support this new class of innovators whose products will make a profound impact in the management and care of pediatric heart patients,” said Additional Ventures CEO Kristie Keller, Ph.D. “We welcome them to our growing community of inventors, researchers and clinicians, and we look forward to working together with our awarded teams and ADPI to bring these products to market. We hope that this competition both inspires and activates the community and brings much-needed new entrants and new ideas to pediatric-first device development.”

APDI is one of five nonprofit consortia in the FDA’s Pediatric Device Consortia grant program. It receives funding to provide a platform of services, expertise and grants that support pediatric innovators in bringing medical devices to the market that specifically address the unmet needs of children. Led by Children’s National, APDI partners include Johns Hopkins University, CIMIT at Mass General Brigham, Tufts Medical Center, MedStar Health Research Institute, MedTech Color and OrthoPediatrics Corp.

photograph of a heart and stethoscope

Study offers quality of life insights for adults with congenital heart disease

photograph of a heart and stethoscope

This research, published today in JAMA Network Open, marks a significant step forward in making better information available for the 1.5 million adults in the United States who were born with CHD.

For the first time, adults living with congenital heart disease (CHD) now have valuable insights into their long-term quality of life through data from the Congenital Heart Initiative (CHI). CHI is the nation’s first and largest patient-focused registry for adults with CHD and released its first study involving over 4,500 participants from all 50 states.

This research, published today in JAMA Network Open, marks a significant step forward in making better information available for the 1.5 million adults in the United States who were born with CHD.

“Studies like this that leverage actual patient voices and experiences help us get a better sense of how to advise, support and treat people with CHD as they age,” says Anitha John, M.D., Ph.D., director of the Washington Adult Congenital Heart program at Children’s National Hospital and senior author of the study. “Also, researchers get a clearer picture of the questions that need to be answered to make sure they have the best quality of life possible.”

The study also demonstrates two of the most successful models of current promising trends in clinical research:

  • The power of patient engagement throughout the research process, including design and implementation.
  • The impact of team science, highlighting the benefits of partnerships between patients, researchers and clinicians.

Key highlights include:

  • Many participants (88%) reported having one or more additional health issues (comorbidities).
    • 33% had arrhythmias (irregular heartbeat).
    • 35% had mood disorders, including depression or anxiety.
  • Quality of life is good or better for 84% of people who completed quality of life reporting measures, regardless of the type of congenital heart condition.
  • People with more complex congenital conditions were less likely to meet physical activity recommendations — an important finding with immediate impact.

Treatments for children born with congenital heart disease have improved so significantly in the last two decades that life expectancy continues to increase as well.

“There are now more adults living with congenital heart disease than there are children with CHD,” says Scott Leezer, patient co-principal investigator for the Congenital Heart Initiative registry and co-author of the study. “However, a significant gap remains in what we know about the adult CHD population. As an adult CHD patient, I was excited to contribute to creating this registry, bringing more answers to people like me who want to know how our unique hearts impact our bodies and quality of life over time.”

The authors note that the study’s findings and the registry data currently have a few limitations. First, the registry only contains patient-reported outcomes and no clinical data. The first sub-study of the CHI, the CHI-RON study, addresses this challenge by incorporating additional data sources for a subset of consenting CHI participants.

Additionally, recall bias, underlying neurocognitive challenges and survey fatigue, may have limited participation in the CHI to a smaller subset of adults with CHD. Efforts are underway to develop methods for people with congenital heart disease who have neurodevelopmental deficits or other disabilities to engage in the registry.  The CHI is temporarily closed to new registrants as the study team redesigns the study to better align with the needs of the community.

“We are grateful for everyone who joined this registry, answered survey questions and shared their experiences,” says Thomas Carton, Ph.D., chief data officer at Louisiana Public Health Institute and study co-author. “The CHI registry is a big step forward for adults with CHD, but also can serve as a model for how to bring together physicians, researchers and patients as active participants in care, research and advocacy.”

As the registry grows in the future, it will focus on increasing diversity of participants, developing additional partnerships with other organizations, continued innovation in data usage and improved community engagement, all with the goal of guiding future research that will ultimately improve quality of life for all adults with CHD.

doctor listening to child's heartbeat

Earlier detection of cardiometabolic risk factors for kids may be possible through next generation biomarkers

doctor listening to child's heartbeat

The next generation of cardiometabolic biomarkers should pave the way for earlier detection of risk factors for conditions such as obesity, diabetes and heart disease in children.

American Heart Association statement finds potential future measures, reiterates importance of heart-healthy lifestyle from birth through adulthood.

The next generation of cardiometabolic biomarkers should pave the way for earlier detection of risk factors for conditions such as obesity, diabetes and heart disease in children, according to a new scientific statement from the American Heart Association published in the journal Circulation.

“The rising number of children with major risk factors for cardiometabolic conditions represents a potential tsunami of preventable disease for our healthcare system,” says the statement’s lead author Michele Mietus-Snyder, M.D., a preventive cardiologist and clinical research scientist at Children’s National Hospital. “But by the time a child is identified based on today’s clinical biomarkers, it’s often too late to reverse the disease trajectory.”

The big picture

The scientific statement included biomarkers that met three criteria:

  • Early and precise clinical detection of metabolic abnormalities before a child begins to show the current clinical signs such as high body mass index (BMI), blood pressure or cholesterol.
  • Mechanistic intervention targets providing immediate risk measures and giving clinicians new targets to personalize and optimize interventions.
  • Modifiable biomarkers that are capable of tracking progression toward or away from cardiometabolic health.

The statement’s identified biomarkers included measures of:

  • Epigenetic, or environmental, factors
  • Gut microbiome health
  • Small particle metabolites in the body
  • Different types of lipids and their impacts on cell membranes
  • Inflammation and inflammatory mediators

The authors proposed these biomarkers with the goal of “expanding awareness to include a whole new realm of biomarkers that precede the traditional risk factors we currently rely upon, such as BMI, blood pressure, cholesterol and blood sugar,” says Mietus-Snyder. “Ideally, these new biomarkers will be added to the array of measures used in clinical research to better assess their value for earlier identification and prevention of global patterns of cardiometabolic health and risk.”

Why it matters

The next generation cardiometabolic biomarkers outlined by the authors are all currently used in research studies and would need to be validated for clinical use. However, Mietus-Snyder notes that the data already collected from these biomarkers in research can make a difference in clinical practice by enhancing our understanding of the deep metabolic roots for children at risk.

Evidence reviewed in the statement shows the risk factors children are exposed to, even before birth, can set the stage for cardiovascular and metabolic health across the lifespan.

Interestingly, all the different factors reviewed have been found to alter the functioning of the mitochondria — the complex organelles responsible for producing the energy for the body that every cell and organ system in turn needs to function. Every class of biomarkers reviewed is also favorably influenced by heart-healthy nutrition, a simple but powerful tool known to improve mitochondrial function.

What’s next

Even as the new so-called ‘omic’ biomarkers reviewed in this statement are developed for clinical applications, there are things clinicians can do to optimize them and improve mitochondrial function, according to Mietus-Snyder.

Most important is to strengthen the collective dedication of care providers to removing the barriers that prevent people, especially expecting mothers and children, from living heart-healthy lifestyles.

We have long known lifestyle factors influence health. Even as complicated metabolic reasons for this are worked out, families can reset their metabolism by decreasing sedentary time and increasing activity, getting better and screen-free sleep, and eating more real foods, especially vegetables, fruits and whole grains, rich in fiber and nutrients, with fewer added sugars, chemicals, preservatives and trans fats. Clinicians can work with their patients to set goals in these areas.

“We know diet and lifestyle are effective to some degree for everyone but terribly underutilized. As clinicians, we have compelling reasons to re-dedicate ourselves to advocating for healthy lifestyle interventions with the families we serve and finding ways to help them implement them as early as possible. The evidence shows the sooner we can intervene for cardiometabolic health, the better.”

pregnant woman on couch

The role of pediatric cardiologists in addressing maternal health disparities

pregnant woman on couch

Black women are two to three times more likely to die from pregnancy-related complications. Most of the risk factors for these complications are cardiac in origin and preventable.

Pediatric cardiologists can and should work alongside other specialties to address the epidemic of maternal mortality that disproportionately affects Black women in the United States, says Annette Ansong, M.D., medical director of outpatient cardiology at Children’s National Hospital.

As co-chair of the Women and Children Committee of the Association of Black Cardiologists Inc., (ABC) Ansong says that cardiologists, especially pediatric cardiologists, have a role to play because “before they are Black women, they are Black girls.”

She talked about the impact of these health disparities and how cardiologists can play a role in addressing them at the American Heart Association Scientific Sessions in November 2022.

Why it matters

Dr. Ansong says that Black women are two to three times more likely to die from pregnancy-related complications. Most of the risk factors for these complications are cardiac in origin and preventable. Furthermore, many of these cardiovascular risk factors, such as obesity, start in youth and some even before birth. For example, children of pre-eclamptic moms have a higher risk of cardiovascular disease in the future, too.

How cardiologists can help

Pediatric cardiologists can be more proactive at helping Black girls grow up into strong, healthy Black women by making sure they are heart-healthy from a young age. That includes advocating for exercise, eating well and exploring innovative ways to encourage those habits.

Dr. Ansong says she makes a point to closely follow children based on the heart health history of their mothers—for example, “if mom had pre-eclampsia, I need to be keeping a closer eye on that child’s blood pressure” —to allow for early intervention and potentially prevent some devastating negative outcomes later in life.

Pediatricians and other specialists can also work with maternal-fetal medicine and other specialties to advocate for better tools to monitor women with pre-existing heart-related risk factors. This might include supporting efforts to enhance technology that makes self-monitoring easier, so women can keep closer eyes on their own blood pressure and share it with doctors in between appointments.

Most important, clinicians of all stripes should try to connect with patients to understand who they are, where they come from and how their stories impact their risk factors for health conditions.

The Women and Children’s Committee of the ABC launched the “We Are the Faces of Black Maternal Health” campaign in February 2022. The first-of-its-kind effort featured the stories of ABC members who had direct or indirect experiences with the impacts of maternal health on themselves, their children or someone they knew.

What’s next

The ABC “We Are the Faces of Black Maternal Health” re-launches this February to continue raising awareness but will also emphasize the need for investments in research about the causes of these disparities and possible prevention strategies to protect Black women.

PeriPath surgery

NIH awards $1.8 million to trial pacemaker delivery system for children

PeriPath pacemaker

The PeriPath access port makes it possible for pacing and defibrillating leads to be placed in the smallest children through holes the size of a straw.

A $1.8 million Small Business Innovation Research (SBIR) grant from the National Institutes of Health (NIH) is funding the first clinical trial of a novel device called PeriPath. The device makes it possible for pacing and defibrillating leads (or wires) to be placed in the smallest children through holes the size of a straw, eliminating thoracotomy or sternotomy procedures for children who are too small for transvenous implantation.

Even the tiniest pacemakers and defibrillators on the market today aren’t small enough for infants and young children with heart rhythm abnormalities. Innovating smaller devices, including adapting current technology like the Medtronic Micra for pediatric use, is a good start but won’t be enough to eliminate some of the challenges for these patients. When a newborn or young child needs any pacemaker or defibrillator, they face open chest surgery. Their arteries and veins are just too small for even the smallest size transvenous pacemaker catheter.

The research goal

Charles Berul, M.D., division chief of Cardiology and co-director of the Children’s National Heart Institute, partnered with engineers in the Sheikh Zayed Institute for Pediatric Surgical Innovation at Children’s National Hospital to develop and test a first-of-its-kind minimally invasive pericardial access tool. The tool allows doctors to place pacing and defibrillation leads to the epicardial surface of the heart under direct visualization from an endoscope.

The team hypothesizes that this tool will allow for pacing and defibrillation therapy to be delivered through a single small port inserted through the skin that is about the size of a drinking straw.

Why it matters: Less pain, shorter and fewer surgeries

If successful, the device will eliminate the need for open chest surgery in patients who aren’t candidates for transvenous placement. The ability to place these leads percutaneously should:

  • Reduce pain and infection risk.
  • Decrease procedure times.
  • Minimize surgery complications that arise from open surgery.
  • Improve better visualization for pericardial punctures.
  • Allow other novel therapies such as epicardial ablation or, in the future, even drug/gene delivery into the pericardial space.

Any implanted pacemaker or defibrillator must be replaced every 5-10 years. A young child in critical need of such devices could face surgeries 10 or more times to replace the device and/or leads.

Pre-clinical testing shows early data that this percutaneous approach is as safe and effective as an open surgical technique, although it remains in early-stage evaluation.

What’s next

The NIH SBIR funding will allow the research team to assess long-term safety and efficacy and commercialize the PeriPath tool. Next steps are to:

  • Refine the design of PeriPath for production manufacturing, integrate testing protocols into a Quality Management System and conduct a pilot verification build. Success is defined as manufacturing production devices that pass 510(k) verification and validation testing.
  • Demonstrate substantial equivalence to predicate trocars through performance and handling validation testing using PeriPath to implant an epicardial lead in a pediatric simulator. If successful, the team will demonstrate equivalence and obtain investigational device exception (IDE).
  • In the latter part of the plan, to perform a first in human feasibility clinical study using PeriPath to implant a commercial pacemaker lead with institutional review board (IRB) approval in infants at Children’s National.

Bottom line

Dr. Berul says, “This research could have a transformative impact on current clinical practice by converting an open surgical approach to a minimally invasive percutaneous procedure.”

He also notes that while the study design focuses on the unique needs of infants and children with congenital heart disease – who are the primary focus of the device – the results of the trial may benefit thousands of adult patients who need pacemakers or defibrillators but who are not candidates for the transvenous placement.

Paper cutout of head with brainwaves

Lifesaving ICDs can cause anxiety, stress, PTSD for parents and kids

Paper cutout of head with brainwaves

Research shows that children with implantable cardioverter-defibrillators, and their parents, are at risk for anxiety, post-traumatic stress disorder and other psychological distress.

Recent advances in design and efficiency of implantable cardioverter-defibrillators (ICDs) have led to their increased use in younger patients, protecting more children with congenital heart disease from sudden cardiac arrest and sudden cardiac death, says a commentary in the journal Heart Rhythm. However, living longer with these devices and the day-to-day worry that they may have to deliver a lifesaving shock in the blink of an eye, may cause unusually high rates of anxiety, stress and other psychosocial distress for children with ICDs and their families.

Commentary authors Vicki Freedenberg, Ph.D., RN, electrophysiology nurse scientist, and Charles Berul, M.D., chief of cardiology, both from Children’s National Hospital, note that current available research shows both children with these ICDs and their parents are at risk for anxiety, post-traumatic stress disorder (PTSD) and other psychological distress. They highlight a new study published in the same journal that reports data related to prevalence and factors associated with PTSD in children with ICDs and their parents as a good start to better understanding these impacts.

Why it matters

Freedenberg and Berul say that the new study adds important information to an area without a lot of previous research. They also point out that understanding the long-term impacts of life with these devices is critical to ensuring the overall long-term health and wellbeing of both the children with these devices and their families.

What’s been the hold-up in the field?

The development of devices that work for younger children with congenital heart disease, including advances in ICDs and pacemakers, has increased in the last decade. In this time, studies of how these devices work for children have focused predominantly on clinical outcomes and questions related to clinical care.

As survival rates for children have increased, research needs to shift from the study of mortality and clinical outcomes toward understanding the full spectrum of how these devices impact daily life for these children and their families.

Moving the field forward

According to Freedenberg and Berul, the new study importantly includes both patient and parent perspectives, which is a first in this research area. They also offer recommendations for future studies, including the use of comparison groups to allow for generalization of findings. Researchers might also ask research questions to determine whether the device itself or the medical and non-medical factors that often occur simultaneously are more important to predicting mental health and wellbeing.

However, the commentary concludes with the most important takeaway: More research, with specific parameters focused on the impact of clinical interventions, is desperately needed to truly understand all the ways that children and their families are affected throughout life by the clinical care and support they receive.

Read the full commentary, Potential for shock leads to potential for stress, in the journal Heart Rhythm.

Baby on ventilator

JAMA study shows no benefit to nitric oxide in cardiopulmonary bypass for young children

Baby on ventilator

An international clinical trial showed that nitric oxide doesn’t help kids recover faster from cardiac surgery with cardiopulmonary bypass.

A study published in JAMA finds that the practice of introducing nitric oxide into the gas flow of the cardiopulmonary bypass oxygenator does not improve recovery or reduce the amount of time a child under age 2 needs to be on a ventilator after cardiac surgery.

Children’s National Cardiac Surgery Chief Yves d’Udekem, M.D., Ph.D., co-authored the international study, which is already leading to changes in how hospitals around the world care for children with congenital heart disease (CHD).

The results are from a double-blind, randomized controlled trial with more than 1,200 participants across six centers in Australia, New Zealand and the Netherlands. The research team found that children under age 2 who had cardiac surgery with cardiopulmonary bypass spent about the same number of days on ventilators after surgery, whether nitric oxide was used during surgery or not.

“These findings do not support the use of nitric oxide delivered into the cardiopulmonary bypass oxygenator during heart surgery,” the authors conclude.

What this means

Previous smaller, single center studies had shown early indications that nitric oxide delivered during heart surgery could possibly improve recovery and shorten the need for respiratory support after surgery by reducing the occurrence of low cardiac output syndrome in children under age 2.

This large-scale international trial showed that this is not the case.

Why it matters

Based on these earlier studies, many hospitals in the United States and around the world who perform critical heart surgery on young children with congenital heart disease had already started to incorporate nitric oxide into cardiopulmonary bypass. This new, more robust data is helping hospitals reassess this practice. Many are stopping it altogether based on the findings.

This work is an important reminder of how valuable well-designed, large-scale, double-blind, randomized, controlled trials are to defining, improving and refining best practices in clinical care.

Also, trials of this size and significance in pediatrics generally, and CHD specifically, take a very long time to complete, if they are ever able to be completed at all. That’s because the number of children with these conditions is relatively small and spread out, even though CHD is the most common birth defect in the world. The authors say it is a major accomplishment to have completed a trial of this size and  in such a short time. Even better, the data gathered from this sample of patients from across international borders can be used to provide even more insights into how best to care for these children as they continue to grow and develop.

Yves D'Udekem

Yves d’Udekem, M.D., Ph.D., named as The Baier Family Distinguished Professor of Cardiac Surgery

Yves d'UdekemChildren’s National Hospital named Yves d’Udekem, M.D., Ph.D., as The Baier Family Distinguished Professor of Cardiac Surgery at Children’s National Hospital.

Dr. d’Udekem serves as chief of cardiac surgery at Children’s National Hospital, co-director of Children’s National Heart Institute, and professor of surgery and pediatrics, The George Washington University School of Medicine and Health Sciences.

About the award

Dr. d’Udekem 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.

Amy and Bret Baier, through their vision and generosity, are ensuring that Dr. d’Udekem and future holders of this professorship will launch bold, new initiatives to rapidly advance the field of pediatric cardiac surgery, elevate our leadership and improve the lifetimes of children with special hearts.

About the donors

The Baiers’ oldest son Paul was born with five congenital heart defects and needed open heart surgery when he was just 12 days old. He has since had three more open heart surgeries and countless procedures and visits to Children’s National. Dr. d’Udekem serves as Paul’s current heart surgeon.

Amy serves as Board Chair of Children’s National Hospital Foundation. Amy and Bret have served numerous times as co-chairs of the Children’s Ball, and Bret has emceed since 2008. They also are co-chairs for follow the leader, the hospital’s comprehensive campaign to fund bold initiatives in pediatric medicine. Children’s National is grateful for the Baiers’ generosity, leadership and commitment.

“I am forever grateful for the Baiers’ generosity and dedication to Children’s National,” said Kurt Newman, M.D., president and CEO of Children’s National Hospital. “With this endowed professorship, Yves will advance the field of pediatric cardiac surgery for kids with special hearts. I am honored to call Amy and Bret friends and partners in pediatric care.”

Anitha John

Youth with heart defects need a smooth transition to age-appropriate heart care, says AHA

Anitha John

Anitha John, M.D., Ph.D., director of the Washington Adult Congenital Heart Program (WACH) at Children’s National Hospital and incoming chair of the AHA Young Hearts council, served as lead author on the statement, which provides the latest evidence-based best practices for a successful transition from pediatric care to adult care.

The American Heart Association (AHA) issued a scientific statement capturing the best practices for helping children with congenital heart disease successfully transition to adulthood and receive health care tailored to their needs as they continue to age. Ensuring a smooth and supported transition and establishing relationships with these young patients as they grow into adults is key to maintaining their engagement and connection to health care decisions that will improve their long-term health and well-being.

Anitha John, M.D., Ph.D., director of the Washington Adult Congenital Heart Program (WACH) at Children’s National Hospital and incoming chair of the AHA Young Hearts council, served as lead author on the statement, which provides the latest evidence-based best practices for a successful transition from pediatric care to adult care. This work is critical given that today, thanks to the tremendous advances in care and treatment of congenital heart defects in the last two decades, there are more adults living with congenital heart conditions than children.

What this means

The AHA describes a scientific statement as an “expert analysis of current research” that “can inform future care guidelines.” This scientific statement demonstrates the scientific evidence supporting what adult congenital heart specialists have advised for years—that making sure children with congenital heart defects continue to stay engaged in their care and actively seek out health care specialized for them as they grow through adolescence and into adulthood is critical. Keeping that connection plays a pivotal role in their overall quality of life as they age.

Why it matters

Treatments and care for children with congenital heart defects has improved so greatly that, according to the AHA, “most people born with heart defects today, including those with complex heart conditions, survive past childhood and become adults.” But the same care they received as children is not enough to address their needs as adults. Adult-oriented congenital heart care can be the difference between a long and healthy life or continued health challenges and dangerous side effects. Adults with congenital heart conditions should seek out care that serves them best, and it should be accessible to everyone who needs it.

By issuing this updated scientific statement, the AHA is broadcasting the important take home message that adults with congenital heart disease and their care providers need (and should seek) access to an adult-focused program with expertise in caring for the unique challenges they face. Establishing that connection at the transition point from adolescence to adulthood can set the stage for long term engagement and health.

Children’s National Hospital leads the way

As director of the WACH program at Children’s National, Dr. John is one of the nation’s experts in care for adults living with congenital heart disease. She also leads significant patient-centered research efforts focused on understanding barriers to care and other challenges faced by these adult heart patients, including serving as co-principal investigator on one of the largest patient-centered studies of adults living with congenital heart disease, supported by the Patient-Centered Outcomes Research Institute (PCORI). The findings from these studies will help fuel further recommendations and guidelines that will improve the standards of care for these patients.

Read the AHA News overview of the Scientific Statement.

Read the Scientific Statement from the Journal of the American Heart Association.

Charles Berul

Charles Berul, M.D., receives Meritorious Achievement Award

Charles Berul

Charles Berul, M.D., chief of Cardiology at Children’s National Hospital has earned a lifetime achievement award, the 2021 Council on Lifelong Congenital Heart Disease and Heart Health in the Young (Young Hearts) Meritorious Achievement Award.

The Meritorious Achievement Award recognizes a person whose achievements have made a significant impact in the field of congenital heart disease and heart health in the young and have helped to further the mission of the Young Hearts council. The council’s mission is to improve the health of children and adults with congenital heart disease and acquired heart disease during childhood through research, education, prevention and advocacy.

Dr. Berul received this award in recognition of his lifelong achievements in the field of pediatric electrophysiology. He is known for his development of innovative electrophysiologic studies for phenotypic evaluations of genetically manipulated pre-clinical models. Over the past two decades, his research focus and passion have been to develop novel minimally invasive approaches to the heart and improving methods for pediatric pacing and defibrillation.

He has also mentored dozens of trainees who have gone on to successful careers and particularly advocates for young investigators and clinician-scientists. He is known for his collaborative style and for supporting advancement of faculty physicians in academic medicine.

Dr. Berul has served on multiple society committees, task forces and writing groups, and is currently an associate editor for the Heart Rhythm Society’s journal. He is also actively involved in other key organizations such as Mended Little Hearts and the Pediatric and Congenital Electrophysiology Society (PACES). He has more than 300 publications and is an invited speaker nationally and internationally in the areas of pediatric cardiac electrophysiology and miniaturized device development.

Dr. Berul received the award on November 12 during a virtual presentation at the American Heart Association’s Scientific Sessions. He is the fourth Children’s National cardiologist to be recognized with this prestigious honor from the council in the last decade.

coronavirus

One-half of MIS-C patients at a single center experienced heart complications

coronavirus

A single center study of patients with multisystem inflammatory disease in children (MIS-C) found that half of children diagnosed with MIS-C had a heart complication as part of the disease. The study collected and analyzed data from 39 cases of MIS-C at Children’s National Hospital in 2020. MIS-C is a pediatric disease that has been linked to SARS-CoV-2, the virus that causes COVID-19.

The study’s findings appear in the journal Cardiology of the Young. The authors aimed to describe the type and frequency of cardiac complications in children with MIS-C while also outlining the disease’s short-term progression. They also hoped to better understand the demographics, clinical and laboratory findings, as well as the therapeutic successes for children with cardiac complications from MIS-C.

“While half of all children at our hospital diagnosed with MIS-C did experience a cardiac complication, it’s important to note that almost all of them (84%) also fully recovered from that cardiac complication within 50 days of diagnosis,” says Ashraf Harahsheh, M.D., director of Quality Outcomes in Cardiology at Children’s National Hospital, who led the study. “We were also able to identify a few common factors among those with cardiac complications that, with further research, may help us identify earlier the children with MIS-C who are at greater risk for heart problems.”

The study found that children with cardiac complications had higher levels of natriuretic peptides, which appear in greater numbers when the heart isn’t pumping enough blood to the rest of the body. Additionally, children who developed heart complications also had higher initial white blood cell counts. MIS-C cardiac complications ranged from mild systolic dysfunction to coronary artery abnormalities and/or artery dilation.

This was a retrospective, observational study of 39 patients admitted to Children’s National Hospital from March 2020 to September 2020 who met the Centers for Disease Control and Prevention MIS-C case definition. Patient demographics, clinical features, laboratory values, diagnostic investigations, including echocardiograms, and therapies were extracted from the electronic medical records.

“This syndrome has some similarities to Kawasaki disease, another inflammatory syndrome that is known to cause cardiac complications,” says Dr. Harahsheh. “Thankfully what we’ve learned from studying and treating Kawasaki disease in children has helped us collaborate with partners around the world to find treatments for MIS-C that seem to minimize the impact of these complications, at least in the short term.”

doctor listening to girl's heart

Decision support tool for chest pain reduces unnecessary cardiology referrals

doctor listening to girl's heart

A new study in the journal Medical Decision Making reports how well a new decision-support tool assisted pediatricians to apply validated criteria and reduce referrals to cardiology for children with chest pain.

In 2017, cardiologists from Children’s National Hospital and other centers published criteria to reliably detect risk for cardiac disease in children presenting with chest pain. However, despite the validated criteria published more than three years ago, as many as half of the children with chest pain who are referred to cardiology from a primary care doctor continue not to meet these criteria.

In response, the cardiology and Children’s National Pediatricians & Associates (CNP&A) team developed a decision support tool based on the validated criteria that was then incorporated into the CNP&A electronic medical record. A study, Promoting Judicious Primary Care Referral of Patients with Chest Pain to Cardiology: A Quality Improvement Initiative, in the journal Medical Decision Making reports how well the tool assisted pediatricians to apply the criteria and reduced referrals to cardiology for children who do not meet criteria for consultation by a pediatric cardiac specialist.

“As stated by the Institute for Healthcare Improvement, improving the U.S. health care system requires simultaneous pursuit of three aims: improving the experience of care, improving the health of populations and reducing per capita costs of health care. Known as the Triple Aim, such improvement includes reducing referrals to specialists for conditions that could be managed in primary care. Fewer unnecessary referrals can reduce costs by decreasing unnecessary testing and specialist time and also has the potential to improve the patient experience by providing care in the medical home,” the authors note.

The study highlights the results of a focused healthcare improvement initiative that engaged pediatricians, nurses, trainees and nurse practitioners at primary care practices to implement the new decision support tool. With the tool in place, the team saw a 71% reduction (from 17% referred to 5% referred) in cardiology referrals for children presenting to cardiology who did not meet the criteria for a referral. At almost one year of follow up, the reduction in referrals based on the criteria did not lead to any missed detections of potential life-threatening events, either.

“This study shows that patients presenting with chest pain who do not meet clinical criteria for referral can be safely and confidently managed at their medical home by their primary care provider,” says Ashraf Harahsheh, M.D., director of Quality Outcomes in Cardiology at Children’s National Heart Institute, who led the study with colleagues. “Avoiding unnecessary referrals to cardiology may help prevent missed work and school days for families and children and will also make sure that the children who truly need a cardiology evaluation can be evaluated quickly.”

This collaboration between our specialty colleagues and primary care clinicians improves care for our patients by bringing an evidence-based approach to managing a condition in a manner that reduces the burden of anxiety for families by addressing their concerns in their medical home,” adds Ellen Hamburger, M.D., study co-author and medical director of the Pediatric Health Network.

After the success of the project at Children’s National Hospital in partnership with the CNP&A, the team is now in talks with UPMC Children’s Hospital of Pittsburgh and Phoenix Children’s Care Network to expand the quality improvement initiative to their primary care networks as well.

Ashraf S Harahsheh, Ellen K Hamburger, Lena Saleh, Lexi M Crawford, Edward Sepe, Ariel Dubelman, Lena Baram, Kathleen M Kadow, Christina Driskill, Kathy Prestidge, James E Bost, Deena Berkowitz. Promoting Judicious Primary Care Referral of Patients with Chest Pain to Cardiology: A Quality Improvement Initiative. Med Decis Making. 2021 Mar 3;272989X21991445. Online ahead of print. DOI: 10.1177/0272989X21991445