cardiology

Scott Klewer, MD, is investigating the molecular pathways directing normal and abnormal heart valve development in order to improve the diagnosis and repair of severe congenital heart defects.

We don't often think of heart disease relating to children, but in the U.S., eight of every 1,000 children born have congenital heart defects. That's 32,000 children each year. At the Steele Center our pediatric cardiologists are taking care of these children's hearts and conducting research to find new and better ways to care for them.

Richard Donnerstein, MD, has developed a new way to determine if an artificial heart valve is working as it should. Artificial valves have been lifesavers for adults and children. "They are very reliable," Dr. Donnerstein says. "One downside is that sometimes we wouldn't know the valve wasn't working until the child had real problems." To avoid that, Dr. Donnerstein has developed a very low-tech, low-cost, but highly effective way to listen to heart sounds. Using digital computer equipment he records the sound of the valve and slows it down. Then it's easy to know the heart valve is working properly. This project is being tested at the Steele Center. Eventually we hope that it will become standard of care and bring peace of mind to parents around the world.

Scott Klewer, MD and PANDA scholar Todd Camenisch, PhD, are working to understand the development of the human heart, to understand at what point the heart can begin to develop abnormally. Of particular concern are babies who are born with Down syndrome as nearly half of these babies also are born with significant heart defects. "This research may pave the way to develop techniques to rescue developing hearts, but that's a long way off", says Dr. Klewer.

Can we change how a heart beats? That's the research question being explored by Ricardo Samson, MD, Children who have slow or irregular heartbeats often require a pacemaker. It's possible that a better understanding of what regulates the heartbeat would allow pediatricians to correct an abnormal heart rate with gene therapy. Dr. Samson is studying the calcium channels, which are the pores by which calcium enters the heart and regulate how much calcium goes in. This, in turn, regulates how hard the heart beats. Researchers believe that the calcium channels are related to the heart's ability to beat spontaneously. If this is so, it's possible that some time in the future, physicians could transfer the calcium channel gene to a heart that doesn't beat properly and restore normal heart function.