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Cardiology

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…

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…

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

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…

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…

Using Whole Exome Sequencing to Find Genetic Cause of Congenital Heart Disease in At-Risk Patients
 Using Whole Exome Sequencing to Find Genetic Cause of Congenital Heart Disease in At-Risk Patients 150 150 Brianne Moore
By: Brianne Moore

Whole exome sequencing has the ability to identify disease-causing mutations, contributing to the development of personalized medicine and bridging a crucial gap between scientific knowledge and clinical application. Congenital heart disease (CHD) is the most common type of birth defect, affecting approximately 40,000 births per year in the United States. While some types of CHD…

For Infants Born Very Preterm, Transcatheter Device Closure of Patent Ductus Arteriosus is Feasible
 For Infants Born Very Preterm, Transcatheter Device Closure of Patent Ductus Arteriosus is Feasible 150 150 Jeb Phillips
By: Jeb Phillips

Optimal timing of procedures and reduction of adverse events are avenues for future study. Approximately half of babies born at less than 32 weeks of gestational age have persistent patent ductus arteriosus (PDA), a condition associated with higher odds of death and respiratory morbidity. When doctors choose to close the ductus, a percutaneous approach is…

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…

Delayed Umbilical Cord Clamping May Benefit Some High-Risk Newborns
 Delayed Umbilical Cord Clamping May Benefit Some High-Risk Newborns 150 150 Jeb Phillips
By: Jeb Phillips

Recent studies show the practice can offer circulatory advantages for infants born extremely preterm or with critical congenital heart disease. The practice of immediate or early umbilical cord clamping after birth has been the norm since research in the 1950s and 1960s showed that most blood volume for full term babies was achieved “within the…

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.…

Cholesterol Screening: What’s the Right Approach?
 Cholesterol Screening: What’s the Right Approach? 150 150 Dave Ghose
By: Dave Ghose

The jury is still out on whether universal testing of children is a good thing. To screen or not to screen? For pediatricians, that question is far from answered when it comes to universal cholesterol testing. Despite an endorsement of the practice from an expert panel at the end of 2011, controversy continues to shroud…

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…

The End of “Under 12″
 The End of “Under 12″ 150 150 Kelli Whitlock Burton
By: Kelli Whitlock Burton

UNOS board eliminates age classification for child lung transplant candidates and alters rules for pediatric heart transplants. Many in the pediatric field were surprised last year when a federal judge ordered that a 10-year-old Pennsylvania girl with cystic fibrosis be added to the national adult waiting list for a double-lung transplant. She had spent 18 months…

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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