Stories about: congenital heart defects

Can we improve neuropsychiatric outcomes in children with congenital heart disease?

Jane Newburger studies neurodevelopment in children with congenital heart defects
Jane Newburger, MD, has dedicated her career to helping children with heart defects reach their full potential.

About 1 out of 100 babies are born with a congenital heart defects. Thanks to medical and surgical advances, these children usually survive into adulthood, but they are often left with developmental, behavioral or learning challenges.

Children with “single-ventricle” defects — in which one of the heart’s two pumping chambers is too small or weak to function properly — are especially at risk for neurodevelopmental problems. “Single-ventricle physiology creates cerebrovascular hemodynamics that can reduce oxygen delivery to the brain,” explains Jane Newburger, MD, MPH, director of the Cardiac Neurodevelopmental Program at Boston Children’s Hospital.

How does this play out in adolescence? In three recent studies, Boston Children’s Heart Center collaborated with the departments of Neurology and Psychiatry to track neurodevelopmental outcomes after corrective Fontan operations. They evaluated preteens and teens as old as 19 — the longest follow-up to date.

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#TBT: How hyperbaric heart surgery saved infants’ lives in the 1960s

Boston Children’s surgical team entering the hyperbaric chamber, loaned from Harvard School of Public Health.
Boston Globe clipping about hyperbaric chamber
From the Boston Sunday Globe, Feb. 10, 1963.

In 1962, the Harvard School of Public Health made a critical loan to Boston Children’s Hospital: the Harvard hyperbaric chamber. It established a new approach to pediatric heart surgery at Boston Children’s.

For many children — including a premature infant named Janet, born in 1964 with a heart murmur — the hyperbaric chamber would prove to be life-saving.

At that time, before the invention of the heart-lung bypass machine, hyperbaric chambers offered a way to operate on infants more safely. That’s because hyperbaric oxygenation, coupled with the effects of increased pressure on the respiratory system, seemed to give infants a better chance of surviving heart surgery.

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First six months of life are best for stimulating child heart growth

heart-regeneration-study2
In these sample sections of mouse heart, the color blue signifies scar tissue. Damage from scarring was minimized by early administration of the drug neuregulin.

Developing a child-centric approach to treating heart failure is no easy task. For one thing, the underlying causes of decreased cardiac function in children vastly differ from those in adults. While most adults with heart failure have suffered a heart attack, heart failure in children is more likely the result of congenital heart disease (CHD), or a structural defect present at birth that impairs heart function. And most therapies designed for adults haven’t proven equally effective in children.

Stimulating heart muscle cells to regenerate is one way cardiac researchers at Boston Children’s Hospital’s Translational Research Center hope to restore function to children’s ailing hearts. In this area, children actually have an advantage over adults: their young heart cells are better suited for regrowth.

Reporting in the April 1 Science Translational Medicine, Brian Polizzotti, PhD, and Bernhard Kuhn, MD, demonstrate that not only does the drug neuregulin trigger heart cell regeneration and improve overall heart function in newborn mice, but its effects are most potent for humans within the first six months of life.

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A portal for beating-heart surgery

Portal for beating heart surgery-analagous to mine entrance

When a patient needs a cardiac intervention, surgeons can choose to access the heart in one of two ways: open-heart surgery or a cardiac catheterization.

Open-heart surgery offers clear and direct access to the heart, but it also requires stopping the heart, draining the blood, and putting the patient on an external heart and lung machine. Catheterization—insertion of a thin, flexible tube through the patient’s groin and up into the still-beating heart—is less invasive. But it’s not suitable for very complicated situations, because it is hard to manipulate the heart tissue with catheter-based tools from such a far distance.

Both methods have been highly optimized, but each has its own risks, benefits and drawbacks. Wouldn’t it be nice if there were a way to directly access the heart and maintain normal heart function and blood flow while repairs are performed?

Nikolay Vasilyev, MD, thought so. A scientist in the cardiac surgery research lab at Boston Children’s Hospital, led by Pedro del Nido, MD, Vasilyev has designed a platform technology that may revolutionize the way we conduct cardiac interventions.

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Sticky heart: Novel surgical glue provides promising alternative for heart procedures

Surgical adhesive interacting with tissue
A close-up view of the adhesive (pink) interacting with collagen tissue (blue). Images courtesy Karp Lab.

A safe and effective adhesive, or glue, that can be used internally in the body has been a pressing need in medicine. Its creation has faced major hurdles—not the least of which is ensuring the glue is nontoxic and capable of repelling fluids—but a new study published today in Science Translational Medicine offers a potential breakthrough.

Congenital heart defects occur in nearly 1 in 100 births, and those that require treatment are plagued with multiple surgeries to deliver or replace implants that do not grow along with the child. Currently, therapies are invasive and challenging due to an inability to quickly and safely secure devices inside the heart. Sutures take too much time to stitch and can cause stress on fragile heart tissue, and the available clinical adhesives are subpar.

“Current glues are either toxic or easily washout in the presence of blood or react immediately upon contacting water,” says Pedro del Nido, MD, chief of Cardiac Surgery at Boston Children’s Hospital and senior co-author of the study. “The available options also tend to lose their sticking power in the presence of blood or under dynamic conditions, such as in a beating heart.”

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Building a whole heart for children born with half: Q+A with a cardiac surgeon

Could a new surgical approach help children like Lucas get the rest of their heart back?

Our pediatric heart surgeons are used to pushing the envelope. Last month we reported on a new kind of heart valve for children with mitral valve defects that can expand as they grow. Now the same team reports 10 years of experience trying to rebuild a lost half of the heart for children born with hypoplastic left heart syndrome (HLHS), a devastating, life-threatening defect.

The new strategy, called staged left ventricle recruitment (SLVR), seeks to harness a child’s native capacity for growth and healing to encourage the undersized left ventricle to grow, giving the child a fully functional heart.

I sat down with Sitaram M. Emani, MD—a cardiac surgeon in the Heart Center at Boston Children’s Hospital and lead author on the SLVR paper—to learn more. 

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New valves for babies that can grow with them

In children with severe mitral valve defects, sometimes valve replacement is the only option. Expandable mitral valves that can be enlarged as a child grows could make caring for such children less complex and invasive.

The human heart is kind of like a busy factory with two powerful pumps—the ventricles—and two “unloading docks,” called the atria. Together, these chambers maintain a delicate balance, ensuring that oxygen-rich blood moves out into the body and that oxygen-poor blood gets pushed back to the heart and lungs.

Just like any factory, however, the heart’s essential functions can be seriously disrupted if just one piece of machinery isn’t working properly.

The mitral valve is a key part of that mechanical balance. This one-way valve helps move blood from the left atrium into the left ventricle, which then pushes the blood out to the body. A failure of the valve can be life-threatening, but fixing or replacing it in children is incredibly complex—and often requires many repeat operations over time.

But two cardiac surgeons at Boston Children’s Hospital, Sitaram Emani, MD, and Pedro del Nido, MD, may have made the repair a little easier by developing a replacement mitral valve that can expand as a child grows.

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