Ebstein's anomaly pediatric research and clinical trials

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Contact the Congenital Heart Valve Program

Pediatric cardiologists and pediatric cardiovascular surgeons at Boston Children's Hospital have pioneered the interventional catheterization techniques now used widely for many congenital heart defects, such as Ebstein’s anomaly.

And a significant amount of the groundbreaking cardiac research currently being conducted at Children’s aims to refine and advance the open heart surgery and catheterization procedures that correct congenital heart defects in newborns and young children—including Ebstein’s anomaly.

Heart valve repair and research

Boston Children’s  Congenital Heart Valve Center cares for children with congenital heart defects that involve absent or malfunctioning heart valves. Our surgeons have a strong record of excellence in heart valve repair and replacement, including minimally invasive techniques.

Our field is rapidly changing. Historically, children who have heart valve conditions have had chronic problems that require lifelong follow-up and treatment. Currently, few artificial replacement heart valves are available that are designed specifically for children, so doctors are limited in their options. Further complicating matters is that when surgeons place a new valve in a child, it doesn’t expand as the child grows, so it needs to be replaced over time.

To address these problems, Boston Children’s cardiac surgery researchers are exploring ways to reconstruct patients’ existing valves. Unlike replacement valves, a reconstructed valve can last a long time and give children an optimal quality of life.

Valve research at Boston Children’s informs our care. Our scientists investigate every aspect of the heart, and the conditions that affect it, so we can offer new and improved treatments to our patients. The primary goal of our research is to combine our clinical and engineering expertise to advance the state of the art in surgical repair of heart valves.

Major themes of our research include:

  • development of novel devices and methods for surgical repair of valves
  • advanced imaging and image processing to better understand and treat diseased valves and to guide minimally-invasive procedures
  • computer simulation of patient-specific valve function to help plan an individual's valve surgery

Cardiac surgery research

Members of Boston Children’s Cardiac Surgery Research Laboratory—a multidisciplinary team of basic and applied research investigators, all of whom hold faculty appointments at Harvard Medical School—are studying the mechanisms of heart disease and new treatments for children with congenital heart defects.

Some principal areas of active research are:

  • surgical robotics and ultrasound-guided intracardiac surgery:The department is pioneering the use of 3-D ultrasound and laparoscopic techniques to operate on the beating heart.
  • myocardial metabolism and myocardial hypertrophy and heart failure:Researchers are exploring new methods of myocardial preservation during heart surgery and the role of angiogenic growth factors in heart failure.
  • tissue engineering to stimulate the growth of new tissue to repair congenital defects, including valve abnormalities, right ventricular defects and arrhythmias

Learn more about Boston Children’s cardiac surgery research initiatives.

Cardiology research

Boston Children’s is a world leader in opening new avenues of translational research—bringing laboratory advances to the bedside and doctor’s office as soon as possible. Senior medical staff members of the Department of Cardiology, who hold faculty appointments at Harvard Medical School—participate in clinical research activities, and many do laboratory research, as well.

Learn more about Boston Children’s current projects in cardiology research.


Cone procedure for tricuspid valve repair

Boston Children’s is one of just a handful of heart centers in the United States that performs the advanced and innovative Cone procedure for repairing the tricuspid valve in children with Ebstein’s anomaly.

In this procedure, extra tissues on the enlarged right side of the heart are folded up, and the malformed valve is reshaped into a cone. (See the Patient story in this topic.)

Creating new ways to perform surgery

Problem: When surgeons perform heart surgery on a baby, they need to open the infant’s chest and stop her heart—an inva­sive, lengthy procedure that can cause life-threatening complications. Recognizing that there is much room for improvement, Pedro del Nido, MD, chief of Cardiac Surgery at Boston Children’s, has developed a novel research program to invent new ways to improve the way that surgeons do cardiac operations.

Innovative solution: Del Nido decided to develop a way to perform surgery on a still-beating heart. But he needed two things that didn’t exist: superior imaging tools that could show the structures inside the heart while it’s beating, and tiny instruments to perform the intricate surgery.

So, he bor­rowed technology from the videogame industry and developed stereo-rendered 3-D ultrasound imaging that allows surgeons to see inside the beating heart as a hologram.

Del Nido also designed new instruments. One is a millimeter-sized tool that extends into the heart through needle-sized incisions. Using a joystick controller and real-time imaging, a surgeon can now navigate through the beating heart’s chambers in animals to remove blockages, repair faulty valves and close leaks.

The other new instrument is a cardioportdevice that allows instruments to be safely introduced into the cardiac chambers with­out the usual risks of blood loss or an air embolism.

cardioportResults: Del Nido’s 3-D tool appears not only to provide superior imaging, but also to yield faster surgery times. Researchers using it to operate on pigs with congenital heart disease performed the procedure 44 per­cent faster than before. Dr. Del Nido’s cardioport will soon be tested in clinical trials and will facilitate further development of similarly novel instruments for heart repair.

Del Nido’s newly-developed cardioport will someday make possible faster, less invasive heart surgery.

History of innovation

In 1938, Children’s cardiac surgeon Robert Gross, MD, performed the world’s first successful surgery to correct a child’s heart defect. Since that time, we have gained recognition around the globe for our leadership in pediatric cardiology and continue to make critical advances in the field. In 2015, U.S. News & World Report named Boston Children’s cardiology and cardiac surgery programs the best of any pediatric hospital in the country.

Trace Boston Children’s history of innovations in pediatric heart care. 

The Cardiac Registry

Before operating on a congenital heart defect, cardiac surgeons need the most precise diagnosis possible. For more than 40 years, the Cardiac Registry at Boston Children’s has been a place where pathologists, cardiologists and cardiac surgeons have gone to learn more about the anatomy of heart defects. Its collection of over 3,600 specimens, some dating back as far as 1944, is irreplaceable, since large, intact examples of heart defects are no longer seen at autopsy in this country. Today, the Registry has been broadened to include developmental cardiology and molecular biology projects.

We are grateful to have been ranked #1 on U.S. News & World Report's list of the best children's hospitals in the nation for the third year in a row, an honor we could not have achieved without the patients and families who inspire us to do our very best for them. Thanks to you, Boston Children's is a place where we can write the greatest children's stories ever told.”
- Sandra L. Fenwick, President and CEO

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