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The Heart Center

Cardiomyopathy in Mothers of Boys With Muscular Dystrophy
 Cardiomyopathy in Mothers of Boys With Muscular Dystrophy 1024 670 Mary Bates, PhD
By: Mary Bates, PhD

Female carriers of the genetic defect that causes muscular dystrophy in boys show evidence of cardiac fibrosis. An interdisciplinary team from Nationwide Children’s Hospital reports about half of women who carry the genetic defect responsible for muscular dystrophy show evidence of cardiac fibrosis. The study, published in the International Journal of Cardiology, suggests that cardiac muscle,…

CMS Approves New Code for Adult Congenital Heart Disease Subspecialty
 CMS Approves New Code for Adult Congenital Heart Disease Subspecialty 1024 683 Katie Brind'Amour, PhD, MS, CHES
By: Katie Brind'Amour, PhD, MS, CHES

The official billing code will enable growth of the subspecialty and is expected to result in improved patient care. In March, the Centers for Medicare and Medicaid Services (CMS) approved a unique code for subspecialists in adult congenital heart disease (ACHD). The code will allow board-certified ACHD specialists to bill as such, rather than as…

COVID-19 in Children With Congenital Heart Disease
 COVID-19 in Children With Congenital Heart Disease 1024 683 Mary Bates, PhD
By: Mary Bates, PhD

Data are still emerging, but COVID-19 – and the subsequent inflammatory syndrome sometimes seen in children – could have serious cardiac impacts. In a recent review article, researchers from institutions including Nationwide Children’s Hospital outlined the impact of COVID-19 in pediatric patients with heart disease. The review, published in the Journal of the American Heart Association,…

From Expectant Parents to Advocates: One Family’s Journey With Hypoplastic Left Heart Syndrome
 From Expectant Parents to Advocates: One Family’s Journey With Hypoplastic Left Heart Syndrome 1024 575 Abbie Miller
By: Abbie Miller

Mila’s parents wanted her to have the best chance at surviving her high-risk comprehensive stage 2 surgery – their journey led them to The Heart Center at Nationwide Children’s. Mid-way into their pregnancy, Jacob and Kayla went in for their 20-week ultrasound. “We were very excited to get into things like paint colors for our…

Technical Aspects of Fetal Aortic Valvuloplasty Predict Success of the Procedure
 Technical Aspects of Fetal Aortic Valvuloplasty Predict Success of the Procedure 1024 575 Mary Bates, PhD
By: Mary Bates, PhD
Fetus in utero receiving valvuloplasty

The procedure is often successful but comes with significant risks to fetal health and life. Fetal aortic valvuloplasty (FAV) is a procedure performed mid-gestation in a subset of fetuses with aortic stenosis who would likely develop hypoplastic left heart syndrome with no intervention. Although often successful, it is a high-risk procedure that is associated with…

Study Supports Expanded Use of Ventricular Assist Devices in Children With Complex Congenital Heart Disease
 Study Supports Expanded Use of Ventricular Assist Devices in Children With Complex Congenital Heart Disease 150 150 JoAnna Pendergrass, DVM
By: JoAnna Pendergrass, DVM

New multicenter study shows that the HeartMate3 ventricular assist device is associated with low morbidity and adverse events in pediatric patients with complex CHD. Ventricular assist devices (VADs), such as the HeartMate3 (HM3) continuous-flow VAD, provide critical heart function support in patients with advanced heart failure. Although design improvements in VADs have reduced complication rates…

New Application of Nuclear Imaging in the Legs May Enable Early Detection of Cardiovascular Problems
 New Application of Nuclear Imaging in the Legs May Enable Early Detection of Cardiovascular Problems 150 150 Katie Brind'Amour, PhD, MS, CHES
By: Katie Brind'Amour, PhD, MS, CHES

Researchers have used a non-invasive imaging modality to quantify perfusion reserve in specific muscles of the extremities — a novel approach with numerous potential clinical applications Researchers have shown for the first time that SPECT/CT imaging can be used to quantify perfusion reserve of specific muscle groups in the lower limbs, which they directly related…

For Some Fetuses With HLHS, One Follow-up Echocardiogram May Be Enough
 For Some Fetuses With HLHS, One Follow-up Echocardiogram May Be Enough 1024 575 Kevin Mayhood
By: Kevin Mayhood

“Not every patient with hypoplastic left heart syndrome, also known as HLHS, is the same: there appears to be a high-risk group that has significant atrioventricular valve regurgitation, atrial septal restriction or other concerns; and a low-risk group that, on their first scan, has none of these comorbidities of the heart,” says Bernadette Richards, FASE,…

Percutaneous Occlusion Linked to Respiratory Improvements in Low-Weight Infants with PDA
 Percutaneous Occlusion Linked to Respiratory Improvements in Low-Weight Infants with PDA 1024 575 Abbie Miller
By: Abbie Miller
Nurse caring for infant in Neonatal Intensive Care Unit (NICU)

Percutaneous occlusion for patent ductus arteriosus (PDA) in infants weighing less than 6 kg is associated with potential longer term improvements in respiratory health, research by investigators at Nationwide Children’s Hospital shows. “A new device for PDA closure recently approved by the FDA has potential to reduce the incidence of device-related complications. This important work…

When is Palliative Care Right for Families of Children With Single Ventricle Congenital Heart Defects?
 When is Palliative Care Right for Families of Children With Single Ventricle Congenital Heart Defects? 150 150 Kevin Mayhood
By: Kevin Mayhood

Practitioners suggest involving a palliative care team early after diagnosis Single ventricle congenital heart defects (SV) disrupt critical blood flow and require a series of complex operations in order for a child to survive. As few as 80% of children born with SV live to age 1, 70% to age 20. Because of the high…

Regular Physical Activities Tied to Improved Quality of Life in Adults With Fontan Circulation
 Regular Physical Activities Tied to Improved Quality of Life in Adults With Fontan Circulation 150 150 Kevin Mayhood
By: Kevin Mayhood

Starting regular exercise in childhood can help lay the groundwork for greater health and quality of life, pediatric cardiologists suggest. Children and adults with Fontan circulation are at risk of a sedentary lifestyle. This is due in part to physicians who advise patients restrict exercise out of concern vigorous physical activity may contribute to venous…

Cheaper, Generic Drug Just as Effective in Preserving Heart Function in Duchenne Muscular Dystrophy
 Cheaper, Generic Drug Just as Effective in Preserving Heart Function in Duchenne Muscular Dystrophy 150 150 Mary Bates, PhD
By: Mary Bates, PhD

A new clinical trial found a cheaper generic drug is effective in protecting the heart in boys with Duchenne muscular dystrophy. A recent clinical trial conducted found a cost-effective generic medication works just as well as a more expensive drug in preserving heart function in boys with Duchenne muscular dystrophy. Published in the Journal of the…

New Model Provides Novel View of Congenital Heart Disease
 New Model Provides Novel View of Congenital Heart Disease 150 150 Lauren Dembeck
By: Lauren Dembeck

The small animal model helps researchers to interpret genomic findings. Researchers at Nationwide Children’s Hospital have developed the first mouse model of congenital heart valve disease using a human gene carrying a disease-causing mutation. Using this model, they were able to follow the human valve disease phenotype from birth to adulthood and identify developmental deficits…

Using Computer Models to Predict How Tissue Engineered Vascular Grafts Will Work
 Using Computer Models to Predict How Tissue Engineered Vascular Grafts Will Work 150 150 Kevin Mayhood
By: Kevin Mayhood

Model and experimental data strongly suggest testing TEVGs until scaffold has biodegraded Identical tissue engineered vascular grafts (TEVGs) being tested in small-diameter veins and arteries of a mouse model performed well for 12 weeks. At 14 weeks, all TEVGs in the veins continued performing well, but all in the arteries suddenly failed. During their effort to understand…

Optimal Use of an FDA-Approved Device for PDA Closure in Infants
 Optimal Use of an FDA-Approved Device for PDA Closure in Infants 150 150 Mary Bates, PhD
By: Mary Bates, PhD

Randomized, controlled clinical trials are needed to answer questions regarding when and with whom to use the device. In January 2019, the US Food and Drug Administration approved a novel device for patent ductus arteriosus (PDA) closure in infants. The Amplatzer Piccolo Occluder is indicated for catheter-based closure of PDA in infants weighing more than…

Variation in Functional Measurements of the Left Ventricle
 Variation in Functional Measurements of the Left Ventricle 150 150 Mary Bates, PhD
By: Mary Bates, PhD

Data from The Pediatric Heart Network Normal Echocardiogram Database show interobserver variability, especially in younger and smaller patients. Echocardiography is the primary tool clinicians have to assess ventricular size and function. Accurate evaluation of the left ventricle is critical in the treatment of patients with congenital and acquired heart disease, as well as other conditions…

Use of Cardiac MRI to Monitor Medical Treatment of Rhabdomyoma
 Use of Cardiac MRI to Monitor Medical Treatment of Rhabdomyoma 150 150 Mary Bates, PhD
By: Mary Bates, PhD

Case study shows success for medical therapy and MRI monitoring of a cardiac rhabdomyoma. In a new case study, researchers from Nationwide Children’s Hospital report treating cardiac rhabdomyoma in a newborn with an mTOR inhibitor called everolimus. They monitored the tumor’s regression using cardiac MRI. Cardiac rhabdomyoma is a common type of heart tumor in…

Fetal Balloon Aortic Valvuloplasty for Critical Aortic Stenosis
 Fetal Balloon Aortic Valvuloplasty for Critical Aortic Stenosis 1024 575 Abbie Miller
By: Abbie Miller
Fetus in utero receiving valvuloplasty

Some heart defects, such as aortic stenosis can be detected on fetal ultrasound. For some fetuses, an intervention can be beneficial before birth. Aimee Armstrong, MD, director of Cardiac Catheterization and Interventional Therapies at Nationwide Children’s Hospital, performs fetal balloon aortic valvuloplasty among other fetal heart catheterization procedures as part of the Congenital Heart Collaborative, a…

Keeping Young Patients With Congenital Heart Disease Connected to Care
 Keeping Young Patients With Congenital Heart Disease Connected to Care 150 150 Mary Bates, PhD
By: Mary Bates, PhD

Race, type of insurance and severity of disease affect likelihood of experiencing a lapse in care for CHD patients younger than 5 years. Lapse in care is prevalent among congenital heart disease survivors by age five, with nonwhites demonstrating elevated risk, according to a new study from Nationwide Children’s Hospital researchers. Medicaid patients and those…

Influences of Maternal Diabetes on Fetal Heart Development
 Influences of Maternal Diabetes on Fetal Heart Development 150 150 Abbie Miller
By: Abbie Miller

Opening the Door to Adult Medicine
 Opening the Door to Adult Medicine 1024 575 Katie Brind'Amour, PhD, MS, CHES
By: Katie Brind'Amour, PhD, MS, CHES

Care transition plans aren’t just for kids with congenital conditions anymore. Could your practice benefit from a proactive transition plan for all patients? In the shift from pediatric to adult care, young patients have the responsibility to adjust to a new life of self-management. But health care providers also play an important role in this…

Are We Turning Away Too Many Pediatric Donor Hearts?
 Are We Turning Away Too Many Pediatric Donor Hearts? 150 150 Katie Brind'Amour, PhD, MS, CHES
By: Katie Brind'Amour, PhD, MS, CHES

More than half of all pediatric donor hearts are declined for use each year, despite the fact that many children die waiting for a heart. But why? And what can be done about it?   As many as one in every four infants on the heart transplant list dies awaiting an organ. For older children,…

Bridging the Gap Between the Genomics Lab and the Cardiology Clinic: CardioGX for Phenotype-Driven Variant Analysis
 Bridging the Gap Between the Genomics Lab and the Cardiology Clinic: CardioGX for Phenotype-Driven Variant Analysis 150 150 Abbie Miller
By: Abbie Miller

The cloud-based platform is a free tool that empowers clinicians and fosters collaboration to find new genetic mutations that cause heart disease. Finding ways to support clinicians and researchers in accessing and utilizing genomic data is paramount to incorporating genomics into clinical care. CardioGX (CardioGenomics eXchange commons) is an interactive web application designed to help clinicians and…

Room for Improvement: Heart Catheterization Risks in Young Patients With Pulmonary Hypertension
 Room for Improvement: Heart Catheterization Risks in Young Patients With Pulmonary Hypertension 1024 575 Katie Brind'Amour, PhD, MS, CHES
By: Katie Brind'Amour, PhD, MS, CHES
Illustration of heart, CAVD

Identifying risk factors for cardiac catheterization complications offers physicians a way to improve pulmonary hypertension care. Up until a few years ago, cardiac catheterization in patients with pulmonary hypertension was considered a low-risk procedure for children and young adults. Some of the country’s best physicians had published studies showing very few complications among hundreds of…

From What-if to Widely Available – A Proposed Path for Tissue Engineered Vascular Grafts
 From What-if to Widely Available – A Proposed Path for Tissue Engineered Vascular Grafts 150 150 Abbie Miller
By: Abbie Miller

What if you could utilize tissue engineering, imaging and 3D printing technologies to bring the benefits of a tissue engineered vascular graft to every child who needs one? Children born with a single ventricle heart defect undergo a series of surgeries and procedures to reroute the blood flow through their heart. The Fontan operation is…

The PDA Conundrum
 The PDA Conundrum 150 150 Jeb Phillips
By: Jeb Phillips

A patent ductus arteriosus is associated with increased morbidity and mortality, but common treatments are associated with poor outcomes as well. What is a neonatologist to do? Until the mid-2000s, most neonatologists were pretty sure they knew how to handle a patent ductus arteriosus (PDA) in an infant born preterm – it needed to be…

The National Mortality Rate for the Comprehensive Stage 2 Procedure
 The National Mortality Rate for the Comprehensive Stage 2 Procedure 1024 575 Jeb Phillips
By: Jeb Phillips

A substantial overall U.S. rate, in contrast to the low rates reported by a few single centers such as Nationwide Children’s, shows room for improvement in handling the hybrid approach for left ventricular physiology. Most published outcomes data on the pediatric Comprehensive Stage 2 procedure come from the few institutions routinely using the “hybrid approach”…

A Key to Preventing Stenosis in Tissue-Engineered Vascular Grafts
 A Key to Preventing Stenosis in Tissue-Engineered Vascular Grafts 600 400 Kevin Mayhood
By: Kevin Mayhood
Tissue engineered vascular graft

Seeding a high number of bone marrow mononuclear cells on graft appears to prevent narrowing. Tissue-engineered vascular grafts hold promise for children with congenital heart disease because the grafts, which carry a patient’s own cells, have the potential to grow and regenerate just as the child’s blood vessels do. But over time, some grafts develop…

Building Heart Valves From Extracellular Matrix
 Building Heart Valves From Extracellular Matrix 150 150 Abbie Miller
By: Abbie Miller

Physicians at Nationwide Children’s Hospital describe the use of an extracellular matrix cylindar valve in the mitral position. At The Heart Center at Nationwide Children’s, Patrick McConnell, MD, a pediatric cardiothoracic surgeon, is using an alternative to the traditional cadaveric, mechanical or biological replacement valve. In a recent case report published in Echocardiography, Dr. McConnell and his colleagues describe the…

How Genes and Environment Interact to Raise Risk of Congenital Heart Defects
 How Genes and Environment Interact to Raise Risk of Congenital Heart Defects 150 150 Mary Bates, PhD
By: Mary Bates, PhD

Genetic susceptibility plus maternal hyperglycemia interact to contribute to heart defects during fetal development. Infants of mothers with diabetes have a three- to five-fold increased risk of congenital heart defects. Such developmental defects are likely caused by a combination of genetic and environmental factors. However, the molecular mechanisms by which maternal diabetes disrupts normal heart…

Dopamine: A Rescue Therapy for Fontan Patients With Protein-Losing Enteropathy?
 Dopamine: A Rescue Therapy for Fontan Patients With Protein-Losing Enteropathy? 150 150 Jeb Phillips
By: Jeb Phillips

Patients with quickly worsening PLE saw serum albumin levels stabilize after dopamine treatment. Patients who have undergone the Fontan procedure for single ventricle congenital heart disease do not often develop protein-losing enteropathy (PLE) – but those who do are at substantial risk of mortality and morbidity. Only heart transplant has been shown to alleviate PLE…

Family Focus Restructures Single Ventricle Care
 Family Focus Restructures Single Ventricle Care 150 150 Brianne Moore
By: Brianne Moore

Standardize, connected care in a multidisciplinary team environment aims to improve outcomes and quality of life for patients born with single ventricle heart anatomy. Children with single ventricle anatomy, one of the most harrowing of congenital heart defects, face unique challenges that require multiple procedures and hospital admissions to treat. In most institutions, each admittance…

Taking Innovation to Heart: Next Gen Interventions in Heart Valve Disease
 Taking Innovation to Heart: Next Gen Interventions in Heart Valve Disease 1024 575 Abbie Miller
By: Abbie Miller

From bioengineers to interventional cardiologists, molecular biologists to cardiothoracic surgeons, experts with diverse backgrounds are focusing on the problem of heart valve disease in children. Heart valve disease affects more than 5 million Americans. And while acquired disease in the adult population certainly accounts for much of this, children with heart valve disease face multiple…

A Narrow Focus: Perfecting Tissue Engineered Vascular Grafts
 A Narrow Focus: Perfecting Tissue Engineered Vascular Grafts 1024 575 Abbie Miller
By: Abbie Miller
image of heart with fontan conduit highlighted

A pair of surgeon-researchers is perfecting tissue engineered vascular grafts through bench, clinical and computational modeling studies.

ANH Preserves Platelet Count and Function Regardless of Storage Method and Time
 ANH Preserves Platelet Count and Function Regardless of Storage Method and Time 150 150 Kevin Mayhood
By: Kevin Mayhood

Length of storage during cardiac surgeries appears to have no detrimental effects on blood. Across the United States, transfusions are standard in cardiac operating rooms, but at Nationwide Children’s Hospital, a practice geared toward limiting blood transfusion has been established and finely honed. According to a recent study from The Heart Center at Nationwide Children’s, more than…

How a QI Project Dramatically Increased Pediatric Survival After Cardiopulmonary Arrest
 How a QI Project Dramatically Increased Pediatric Survival After Cardiopulmonary Arrest 150 150 Jeb Phillips
By: Jeb Phillips

A survival rate of 37-48 percent among young heart surgery patients who experience code events is the norm. A multidisciplinary review process helped achieve an 81 percent rate at Nationwide Children’s Hospital. Survival rates have remained stubbornly low for young heart surgery patients who experience cardiopulmonary arrest; critical care literature reports that fewer than 50…

Understanding Stress Experienced by Survivors of Congenital Heart Disease
 Understanding Stress Experienced by Survivors of Congenital Heart Disease 150 150 Tiasha Letostak, PhD
By: Tiasha Letostak, PhD

For adolescent and adult survivors of congenital heart disease, how they perceive disease-related stress has a key connection to how they report health outcomes, such as quality of life and emotional well-being. Researchers at Nationwide Children’s Hospital are the first to systematically identify different types of disease-related stress experienced by survivors of congenital heart disease,…

A New Use for Kangaroo Care
 A New Use for Kangaroo Care 150 150 Kevin Mayhood
By: Kevin Mayhood

A parent’s touch reduces agitation, allows infants and toddlers to remain extubated after heart surgery. A small percentage of babies and young children who have undergone congenital cardiac surgery and early tracheal extubation are treated with a calming parent’s touch at Nationwide Children’s Hospital – a strategy that physicians have found works better in some…

Signaling Pathway Changes May Flag CAVD, Offer Target for Therapies
 Signaling Pathway Changes May Flag CAVD, Offer Target for Therapies 150 150 Kevin Mayhood
By: Kevin Mayhood

Loss of TgfB1 from the endothelium leads to a reduction in Sox9 expression and valve calcification. A team of researchers has identified a molecular signaling pathway that, when altered, can contribute to calcific aortic valve disease (CAVD). The finding may provide a method for early diagnosis — many patients don’t learn they have the disease until it’s in the final…

The Transfusion Evolution
 The Transfusion Evolution 576 367 Abbie Miller
By: Abbie Miller

The first successful open heart surgery with cardiopulmonary bypass was performed in 1953, but it wasn’t until the 1970s that these surgeries began to have high success rates — due in large part to the availability of fresh whole blood transfusions. However, fresh whole blood is difficult to attain. In response, blood component transfusions became…

InSight: A Window to the Heart
 InSight: A Window to the Heart 720 530 Katie Brind'Amour, PhD, MS, CHES
By: Katie Brind'Amour, PhD, MS, CHES

Cardiac MRI for Hypoplastic Left Heart Syndrome Hypoplastic left heart syndrome (HLHS) is a potentially fatal congenital heart defect affecting blood flow. It requires multiple palliative surgeries, starting within the first week of life. Nationwide Children’s employs a hybrid surgical approach. Hybrid Stage I (not shown) Bilateral branch pulmonary bands are placed to restrict blood flow.…

Survivors of Congenital Heart Disease Report Poor Risk Knowledge and High-Fat Diets
 Survivors of Congenital Heart Disease Report Poor Risk Knowledge and High-Fat Diets 150 150 Tiasha Letostak, PhD
By: Tiasha Letostak, PhD

A new study suggests that CHD survivors may lack knowledge about their disease and would benefit from education about future risks and health behaviors. Over 1 million adults with congenital heart disease (CHD) currently reside in the United States. These individuals are at heightened risk for numerous health concerns, including endocarditis, pregnancy complications and life-threatening…

Adult Congenital Heart Disease: Let’s Finish What We Started
 Adult Congenital Heart Disease: Let’s Finish What We Started 150 150 Curt Daniels, MD
By: Curt Daniels, MD

Congenital heart disease is the most common birth defect, diagnosed in nearly 1 percent of all births in the United States. Traditionally, life expectancy in many infants with severe CHD was limited to months. However, advances in medical and surgical care have led to remarkable improvements in survival: the median age of those living with severe…

By the Book: Anticoagulation for CHD
 By the Book: Anticoagulation for CHD 150 150 Kelli Whitlock Burton
By: Kelli Whitlock Burton

The American Heart Association (AHA) has released its first evidence-based guidelines on anticoagulation in congenital heart disease (CHD). Anticoagulation is a key element in managing patients with congenital heart disease. Despite the therapy’s widespread use, there are no established guidelines on anticoagulants in this patient population, which often leads to inconsistency and guesswork in how…

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