Cardiology

Stem Cell Study Opens Door to Understanding Development of Rare Form of Congenital Heart Disease
Stem Cell Study Opens Door to Understanding Development of Rare Form of Congenital Heart Disease 150 150 Pam Georgiana

Researchers use induced pluripotent stem cell technology and single-cell genomics to pinpoint abnormal cell development in hypoplastic right heart syndrome. A rare form of hypoplastic right heart syndrome (HRHS), pulmonary atresia with intact ventricular septum (PA-IVS), occurs when the structures on the right side of the heart are malformed. Specifically, in PA-IVS, the pulmonary valve…

Acute Kidney Injury After Comprehensive Stage Two Palliation in Infants With Hypoplastic Left Heart Syndrome
Acute Kidney Injury After Comprehensive Stage Two Palliation in Infants With Hypoplastic Left Heart Syndrome 150 150 JoAnna Pendergrass, DVM

Infants with hypoplastic left heart syndrome who undergo the Hybrid pathway palliation commonly develop acute kidney injury after the comprehensive stage 2 procedure and may require advanced medical therapy.   In a recent Cardiology in the Young publication, Andrew Yates, MD, associate medical director for the Cardiothoracic Intensive Care Unit at Nationwide Children’s Hospital, and…

Implanted Hemodynamic Monitoring in Patients With Fontan Circulation
Implanted Hemodynamic Monitoring in Patients With Fontan Circulation 150 150 Mary Bates, PhD

Pulmonary artery pressures obtained with the CardioMEMSTM system may be clinically useful. In a new study, researchers from The Ohio State University and Nationwide Children’s Hospital report on the use of invasive implanted hemodynamic monitoring (IHM) in patients with Fontan circulation. The findings suggest that IHM pressures in these patients are more closely associated with…

Researchers Create a Stem Cell Line to Study Alagille Syndrome
Researchers Create a Stem Cell Line to Study Alagille Syndrome 1024 683 Mary Bates, PhD

The patient-derived iPSCs have a mutation affecting the Notch signaling pathway. Researchers at Nationwide Children’s have developed an induced pluripotent stem cell (iPSC) line derived from a patient with Alagille syndrome, a multisystem genetic disease. Like the majority of patients with Alagille syndrome, the stem cells have a pathogenic mutation in the JAG1 gene, part…

Congenital Heart Disease and Extracardiac Phenotypes Associated With Novel Pathogenic Variant in GATA6
Congenital Heart Disease and Extracardiac Phenotypes Associated With Novel Pathogenic Variant in GATA6 1024 575 Jessica Nye, PhD

A study published in Pediatric Research describes a novel pathogenic variant in the gene GATA binding protein 6 (GATA6) associated with congenital heart disease (CHD), diabetes mellitus and necrotizing enterocolitis.   GATA is a family of transcription factors that encode zinc finger DNA binding proteins which are critical regulators during organogenesis. Pathogenic variants in three…

Ensuring Vulnerable Babies Receive the Benefits of Human Milk
Ensuring Vulnerable Babies Receive the Benefits of Human Milk 1024 575 Mary Bates, PhD
small baby with nose canula

A QI project increases human milk consumption among newborns hospitalized with congenital heart disease. The American Academy of Pediatrics and the World Health Organization recommend human milk for infants because of its important health benefits. However, most newborns with congenital heart disease (CHD) are separated from their mothers shortly after birth and admitted to a…

Counseling Families of Patients with Hypoplastic Left Heart Syndrome
Counseling Families of Patients with Hypoplastic Left Heart Syndrome 150 150 Mary Bates, PhD

A new survey investigates how palliation strategies have changed in the last ten years. Approximately ten years ago, researchers at Nationwide Children’s Hospital surveyed pediatric cardiac providers regarding initial counseling for families of patients with hypoplastic left heart syndrome (HLHS). Given the evolution of options and outcomes since that time, the team recently queried providers…

Changing the Odds: Survival Trends in Children With Down Syndrome and Congenital Heart Defects
Changing the Odds: Survival Trends in Children With Down Syndrome and Congenital Heart Defects 1024 683 Pam Georgiana
Smiling baby with Down syndrome

About half of the children born with Down syndrome also have congenital heart defects (CHD). As a transplant cardiologist, Lydia K Wright, MD, wanted to learn if CHD was still the most significant mortality risk for these patients before age five. [1] “Our aim was to assess whether the survival rate of kids with Down…

Tissue Engineering and Fetal Medicine: A New Frontier for Congenital Heart Disease
Tissue Engineering and Fetal Medicine: A New Frontier for Congenital Heart Disease 1024 575 Abbie Miller
Fetus in utero receiving valvuloplasty

Procedures that utilize cardiac catheterization to improve fetal heart development are often successful, but they are not without risk. And even if they can successfully prevent the development of single ventricle disease, there is always ongoing heart valve disease (HVD). HVD may not be as life-threatening as single ventricle disease, but it requires lifelong care.…

Susceptibility to and Use of E-cigarettes and Marijuana Is Common Among Adolescents With Congenital Heart Disease
Susceptibility to and Use of E-cigarettes and Marijuana Is Common Among Adolescents With Congenital Heart Disease 1024 575 Lauren Dembeck

Adolescents with congenital heart disease (CHD) are subject to disease-related stressors, such as attending medical appointments and undergoing medical procedures. They have elevated risk for cardiovascular and cognitive complications, which may be exacerbated by the use of e-cigarettes and marijuana. To inform prevention strategies for their long-term wellbeing, it is critical to understand patterns of…

Using Machine Learning in the Electronic Medical Record to Save Lives
Using Machine Learning in the Electronic Medical Record to Save Lives 1024 683 Abbie Miller
physicians in white coats looking at a tablet

The deterioration risk index identifies patients at risk for deterioration and poor outcomes, triggering the care team to act before a crisis happens. In a report published in Pediatric Critical Care Medicine, a team from Nationwide Children’s Hospital describes a machine learning tool for timely identification of hospitalized children at risk for deterioration – a…

The PROMISE Program Reduces Cardiac Arrests in High-Risk Patients
The PROMISE Program Reduces Cardiac Arrests in High-Risk Patients 600 400 Mary Bates, PhD

This proactive quality improvement initiative improved outcomes for high-risk pediatric cardiology patients undergoing cardiac intervention. Faculty within The Heart Center at Nationwide Children’s recently initiated proactive risk mitigation strategies to reduce post-procedural cardiac arrests in high-risk congenital heart patients. In a new paper, the team used quality improvement methodology to show that their efforts resulted…

Myopericarditis After COVID-19 Vaccination
Myopericarditis After COVID-19 Vaccination 1024 683 Katie Brind'Amour, PhD, MS, CHES

A meta-analysis of international studies offers more detailed insight into the severity and outcomes of vaccine-related myopericarditis in the adolescent and young adult population. Concerns over myopericarditis and other cardiovascular complications in teens and young adults have gained considerable media attention. While myopericarditis-related data have been well characterized in adults with and without vaccination, the…

Study Identifies Multiple Cell Signaling Pathways for Calcification in Aortic Valve Disease Regulated by Nitric Oxide
Study Identifies Multiple Cell Signaling Pathways for Calcification in Aortic Valve Disease Regulated by Nitric Oxide 1024 753 Lauren Dembeck

Exploring novel signaling mediators in calcific aortic valve disease may pave the way for the development of pharmacotherapies to treat this intractable condition during early stages of its progression.   Calcific aortic valve disease is a chronic inflammatory condition characterized by progressive fibrosis and calcification of the heart valves, which can cause narrowing of the…

Celebrating More Than 100 Heart Transplants — And a Host of Other Heart Program Achievements
Celebrating More Than 100 Heart Transplants — And a Host of Other Heart Program Achievements 1024 746 Deipanjan Nandi, MD, MSc

Deipanjan Nandi, MD, MSc, shares an update about treating heart failure at Nationwide Children’s Hospital. From transplant to ventricular assist devices and pharmaceutical options, the patient-centered care offers innovative options for patients with heart failure.

Researchers Characterize Growth and Remodeling of Tissue-Engineered Blood Vessels
Researchers Characterize Growth and Remodeling of Tissue-Engineered Blood Vessels 600 400 Lauren Dembeck
Tissue engineered vascular graft

Tissue engineering may soon give babies with congenital heart disease new blood vessels capable of native function and growth.   Major cardiovascular reconstructive operations in babies with congenital heart defects require the use of artificial blood vessels called vascular grafts. Because these grafts are made of man-made materials, the recipients’ tissues eventually begin to outgrow…

Variability in Cardiac Diagnostics and Therapies for Patients with Duchenne Muscular Dystrophy
Variability in Cardiac Diagnostics and Therapies for Patients with Duchenne Muscular Dystrophy 1024 670 Mary Bates, PhD

Survey shows different approaches to cardiac diagnostics and therapy among a multicenter collaborative network. Cardiac disease is a major cause of mortality in Duchenne muscular dystrophy (DMD), yet data regarding outcomes and cardiac disease progression are lacking. This has led to a highly variable approach to heart failure therapy in DMD from center to center,…

Multi-Disciplinary Team Develops Evidence-Based Guidelines for Acute Management of Persistent Pulmonary Hypertension in Newborns
Multi-Disciplinary Team Develops Evidence-Based Guidelines for Acute Management of Persistent Pulmonary Hypertension in Newborns 200 133 Katie Brind'Amour, PhD, MS, CHES

What began as an internal effort to simplify practice has transformed into published evidence-based guidelines to standardize care for acute stabilization and management of newborns with pulmonary hypertension.   Persistent pulmonary hypertension of the newborn (PPHN) is a highly varied and changeable condition that presents a challenge for clinical management, even among tertiary care centers…

Researchers Classify High-Risk Variants in Lamin A/C Cardiac Disease Using Machine Learning
Researchers Classify High-Risk Variants in Lamin A/C Cardiac Disease Using Machine Learning 1024 575 Lauren Dembeck
conceptual art of DNA

A recent study from researchers at Nationwide Children’s Hospital, published in Heart Rhythm, describes how a LMNA variant of unknown significance was identified as high-risk using family histories, genomic analysis and machine learning. “Genetic variants in LMNA are well known to cause a spectrum of clinical disease — referred to as laminopathies — particularly cardiomyopathy,…

Physical Activity Intervention May Benefit Adolescents With Congenital Heart Defects
Physical Activity Intervention May Benefit Adolescents With Congenital Heart Defects 1024 684 Mary Bates, PhD
teen girl using cell phone

A lifestyle intervention delivered via videoconference increased physical activity in the least active participants. In a new pilot study, researchers from Nationwide Children’s evaluated the efficacy of the Congenital Heart Disease Physical Activity Lifestyle (CHD-PAL) intervention for high school-aged adolescents with congenital heart defects. The results demonstrate the intervention is feasible and acceptable, and preliminary…

First In Human
First In Human 1024 752 Abbie Miller
Top-down view of heart valves

The Heart Center at Nationwide Children’s Hospital has a long history of helping to bring innovations from the lab to the patient. The latest, the Autus Valve, aims to improve care and outcomes for children with pulmonary valve disease. In December 2021, Mark Galantowicz, MD, cardiothoracic surgeon and director of The Heart Center at Nationwide…

Standardizing Training in Pediatric Cardiac Critical Care
Standardizing Training in Pediatric Cardiac Critical Care 150 150 Mary Bates, PhD

In a new report, a taskforce of experts presented their recommendations to standardize training in pediatric cardiac critical care. The authors hope the report is a first step toward more oversight of training and certification in the field. The field of pediatric cardiac critical care is relatively young but growing quickly. Currently, there are 120…

How Patient-Derived Stem Cells are Changing the Trajectory of Congenital Heart Disease Research
How Patient-Derived Stem Cells are Changing the Trajectory of Congenital Heart Disease Research 1024 683 Katie Brind'Amour, PhD, MS, CHES

Small blood samples — and the patient-specific, induced pluripotent stem cells (iPSCs) they enable — turn into valuable research material for understanding congenital cardiovascular disorders, especially when united with modern genome sequencing and editing, animal models and three dimensional tissue growth technologies. Patient-derived induced pluripotent stem cells (iPSCs) have a significant downside for studying most…

Stent Implantations Are an Option for Small Patients
Stent Implantations Are an Option for Small Patients 1024 670 Mary Bates, PhD

Stents that can be dilated to adult size are safe and effective for the treatment of coarctation of the aorta in young patients. Studies have demonstrated stent implantation is more effective and safer than balloon angioplasty for the treatment of coarctation of the aorta in adult and older pediatric patients. In smaller patients, however, particularly…

Occurrence and Cost of Infections After Heart Transplant
Occurrence and Cost of Infections After Heart Transplant 1024 683 Mary Bates, PhD
Dr. Emily Hayes

Researchers identify risk factors, long-term outcomes of vaccine-preventable infections following heart transplantation in pediatric patients. Recently, a study using the Pediatric Health Information System (PHIS) database found that one in six pediatric solid organ transplant recipients were hospitalized with respiratory syncytial virus (RSV) or vaccine-preventable illness in the first five years after transplant. Within this…

How Important is Fasting for Pediatric Routine Cholesterol Screening?
How Important is Fasting for Pediatric Routine Cholesterol Screening? 1024 575 Andrew Tran, MD

A nonfasting lipid panel is a great first-line screening tool to use. While it is ideal to have a fasting lipid panel, this can be difficult to obtain in practice. For the purposes of screening, I think that it is much more important to go ahead and get the nonfasting lipid panel while the patient…

Incidence and Impact of Acute Kidney Injury in Patients With Hypoplastic Left Heart Syndrome
Incidence and Impact of Acute Kidney Injury in Patients With Hypoplastic Left Heart Syndrome 150 150 Abbie Miller

While incidence of severe kidney injury after surgery for hypoplastic left heart syndrome is low, it may be linked to increased 30-day mortality. Acute kidney injury – the abrupt decline of renal function – following pediatric heart surgery leads to worse outcomes, including increased mortality. The overall incidence of acute kidney injury (AKI) after pediatric…

COVID-19 and the Heart: SARS-CoV-2-Associated Myocardial Infection
COVID-19 and the Heart: SARS-CoV-2-Associated Myocardial Infection 150 150 Simon Lee

Myocardial infection following COVID-19 illness has made headlines. Simon Lee, MD, tackles some commonly asked questions about MIS-C and isolated myocarditis after COVID-19 recovery.

Risk Stratification in Infants With Primary Pulmonary Vein Stenosis
Risk Stratification in Infants With Primary Pulmonary Vein Stenosis 1024 681 Mary Bates, PhD
sleeping infant

Categorizing infants with pulmonary vein stenosis into stable and progressive groups could help inform treatment. Primary pulmonary vein stenosis is a rare, often lethal, cardiac disease. It is challenging to treat, as the disease can be progressive in some patients but not others. In a new study, researchers from Nationwide Children’s describe outcomes among preterm infants…

A Collaborative Approach to Preventive Cardiology
A Collaborative Approach to Preventive Cardiology 1024 575 Andrew Tran, MD

Many of us have friends or family members who have had an early heart attack. The event is sudden, unexpected and sobering. Those who recover often make drastic changes to their diet and lifestyle, along with taking medications, and endeavor to delay and undo years of accumulated toll. However, these efforts can only go so…

Cardiomyopathy in Mothers of Boys With Muscular Dystrophy
Cardiomyopathy in Mothers of Boys With Muscular Dystrophy 1024 670 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

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

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,…

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
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

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

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

“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,…

Targeting Cardiac-Related Strokes in Infants
Targeting Cardiac-Related Strokes in Infants 150 150 Mary Bates, PhD

New study describes risk factors for stroke in children with cardiac disease. Advances in surgical techniques and intensive care management have led to an increase in the number of infants surviving with cardiac disease. Unfortunately, children with cardiac disease are at higher risk for experiencing an arterial ischemic stroke. Now, a new study points to…

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

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

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

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

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…

Variation in Functional Measurements of the Left Ventricle
Variation in Functional Measurements of the Left Ventricle 150 150 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

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…

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

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

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

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
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…

Building Heart Valves From Extracellular Matrix
Building Heart Valves From Extracellular Matrix 150 150 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…

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