- The Washington Times - Monday, January 23, 2006


One of medicine’s greatest triumphs is hitting a snag: Up to 1 million people born with once-lethal heart defects have grown up, a pioneering generation largely unaware that heart repairs can wear out as middle age approaches.

Few even get cardiac checkups, apparently thinking they were cured as children — although a surgical repair isn’t a cure. Worse, few cardiologists outside of children’s hospitals have any idea how to care for these special hearts.

“The disease as an adult is completely different than the other kinds of heart disease adults get,” warns Dr. Karen Kuehl, a pediatric cardiologist at Children’s National Medical Center in Washington, who recently helped open one of the nation’s few specialty clinics for adults with congenital heart disease.

“Here are people in their early young adulthood who have thought that they went through this [childhood surgery] … and they were going to be fine. In fact, we don’t know that,” Dr. Kuehl said. “Now we’re seeing things nobody would have predicted.”

Open-heart surgery for babies and young children did not become common until the 1970s. Before then, only a quarter of so-called “blue babies” and other infants born with complex heart defects lived beyond a year. Now, more than 95 percent of these “miracle babies” will grow up, living near-normal lives for many years.

Only recently have enough of the early survivors reached adulthood for doctors to notice a disturbing trend: Starting about 20 years after childhood surgery, the risk for some serious problems — irregular heartbeats, enlarged hearts, heart failure, occasionally even sudden death — begins to rise among people who had complex defects repaired.

If caught early, many such problems are treatable or preventable. Too often, though, patients have serious damage or need a heart transplant by the time someone links their survived birth defect to the new illness, said Dr. Roberta Williams of the American College of Cardiology.

Doctors do not know how big the risk is or who most needs preventative care, although pregnancy demands extra caution. There have been no large-scale studies of survivors’ long-term health, partly because so many disappear once they outgrow the pediatric cardiologist. Less than half of adults with congenital heart disease are thought to receive regular cardiac care.

Denial plays a role. Young adults who feel well, and who no longer are on a parent’s insurance policy, desperately want to be normal and may not see a need for checkups.

Even if they want ongoing care, there are only about 100 cardiologists nationwide specially trained in adult congenital heart disease. Considering that one in 150 babies is born with a heart defect, a soon-to-skyrocket new population of adult patients will compete for limited specialists.

“There’s a setup for people to fall between the cracks,” said Dr. George Ruiz of the Washington Hospital Center, who with Dr. Kuehl runs the new Washington Adult Congenital Heart Center — and is combing through boxes of surgical records dating to the 1970s in hopes of tracking down now-grown “miracle babies” who do not know that their repairs may not last for life.

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