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In the 1980s, at about the same time Dr. Rosenbaum was beginning to specialize in adult congenital heart disease, Michael and Joan Schneeweiss were searching for a cardiologist for their son who had had heart surgery several years earlier and was outgrowing the expertise of his pediatric cardiologists.

"We moved over to the adult cardiology section, but found that the adult cardiologists were not trained to deal with our son's particular heart anatomy," Mrs. Schneeweiss says. "Then we found Dr. Rosenbaum. We were very relieved, because by that point, we didn't know where else to go."

During the next few years, the Schneeweisses – who were also instrumental in founding a parents group, dubbed "Heartbeat Cabaret," in 1986 to raise funds for pediatric cardiology at Babies Hospital – decided to create a center for adults with congenital heart disease at Columbia University.

"The Schneeweisses were very insightful in seeing the growing need for more specialized adult care," Dr. Rosenbaum says. "They recognized that adult patients no longer belonged in a children's hospital waiting room."

To turn their idea into reality, in 1998 Joan and Michael Schneeweiss gave $1 million to Columbia to start the Joan and Michael Schneeweiss Center for Adult Congenital Heart Disease, which Dr. Rosenbaum directs.

Since then, the program has grown tremendously, with patients coming from all over the country to see Dr. Rosenbaum and his colleagues.

The funds have been instrumental in providing the center with its own dedicated equipment and staff to image patients' hearts. "By having these essential services in one location, we can review studies and develop a treatment plan during the same consultation," Dr. Rosenbaum says. "Before we had our center we had to send patients two blocks away to the Children's Hospital for an echocardiogram. It was time consuming and consultations became disjointed."

Says Mrs. Schneeweiss, "We're very happy with the center and how Dr. Rosenbaum has turned it into such a well known locus for patients and their doctors. Our family was very fortunate to have found him at Columbia."

—Susan Conova

Though the first surgery to correct a congenital heart defect was performed in 1944, for decades afterward only a few children born with complex heart defects such as missing valves, or transposed arteries survived past adolescence. This has all changed. Today, an estimated 750,000 adult patients in the United States have congenital heart disease, and their numbers are growing.

Dr. Marlon Rosenbaum, associate clinical professor of medicine and pediactrics and director of the Joan and Michael Schneeweiss Center for Adult Congenital Heart Disease at Columbia, was a fellow studying arrhythmia at Massachusetts General Hospital in Boston in 1985 when he realized he was witnessing the beginning of a new era in congenital heart disease.

"I began to see a small group of adults with congenital heart disease who presented with unique problems," says Dr. Rosenbaum. "I recognized these adults were just the tip of the iceberg and that the vast majority of children with congenital heart disease would now reach adulthood. But their problems were only beginning to be defined."

By the 1980s, hard-won advances in medical and surgical treatment were starting to carry the children into their teens and 20s, but medical care was not keeping pace in its ability to care for adult patients. Most were still being cared for by their pediatric cardiologists' patient's condition.

But as more and more children with congenital defects grew into adulthood, new problems emerged that pediatric cardiologists had not confronted. In part, this may be because children's hearts are smaller and less prone to some of the problems that arise in heart muscle that is more dilated.

While seeing an adult cardiologist seemed logical, most were often not aware of the congenital heart patient's unique anatomy, in the same way that pediatricians were often unfamiliar with the problems of adult heart patients.

"To most adult cardiologists the anatomy of complex congenital heart disease is like a black box," Dr. Rosenbaum says. "Few adult cardiologists received training in congenital heart disease during their fellowships. An adult cardiologist would do an echocardiogram and see connections he or she never saw before. Many adult cardiologists recognized that they were not comfortable treating these types of patients."

Seeing the need for a specialist who could bridge the gap, Dr. Rosenbaum came to Columbia in 1986 to continue his training in arrhythmias and congenital heart disease. In 1987, he joined both the adult and pediatric cardiology staff to develop a program in adult congenital heart disease and began focusing full-time on this disease. Since then, he has built a team of both cardiac and noncardiac specialists who treat the unique and often complicated heart and medical problems of adults. The team includes cardiac surgeons who are experts in congenital heart disease and transplantation surgery; electrophysiologists; interventional cardiologists; and, noncardiac specialists in radiology, gastroenterology, pulmonology, hematology, obstetrics and gynecology. Since 1998, they have been united in the Joan and Michael Schneeweiss Center for Adult Congenital Heart Disease at Columbia (see sidebar).

"Over time, these specialists have grown accustomed to the unique anatomic and hemodynamic issues in our adult congenital heart patients," Dr. Rosenbaum says. "It is extremely important to provide allied care for these patients." In the Schneeweiss Center, specialists strive to find the optimal solution for every patient, especially for the complex arrhythmias that are now one of the adult patient's most significant problems.

"It's not simply prescribing a medication or recommending an ablation to try to control an arrhythmia," Dr. Rosenbaum says. "When the anatomy is normal, it's relatively straightforward to determine where an arrhythmia arises. But in congenital cases, areas of abnormal conduction may be difficult to reach, multiple circuits may be involved rather than only one, and it may not be possible to eliminate the problematic tissue."

In some instances, the doctors have combined interventional procedures in the electrophysiology laboratory with surgery to repair a problem. This approach has been very effective with the use of stents and other devices to approach areas that are hard to reach with surgery.

Increasing the challenge is the fact that every congenital heart patient has a unique medical history.

"Someone born in the 1970s with his aorta and pulmonary artery attached to the wrong chambers, for example, would have had a 'baffle' constructed within the upper chamber of the heart, which creates its own problems later in adulthood," Dr. Rosenbaum says. "On the other hand, someone with the same anatomy born in 1990 would have had the two arteries returned to the appropriate chambers, a procedure that creates an entirely different set of potential issues. Every patient who comes to see us is different."

Dr. Rosenbaum has been enormously successful in expanding the Schneeweiss Center, increasing the number of patients seen there, tackling more complex issues, and consolidating essential services in one location. Still, the need is greater than the availability of specialists.

"We simply do not have enough full time specialists in adult congenital heart disease," Dr. Rosenbaum says. "The challenge will be in deciding how best to train these cardiologists in a way that maintains effective linkages between pediatric and adult cardiology."

Training nonspecialists to be more alert to the needs of adults with congenital heart disease is equally important. "The adult cardiologist often finds himself on the front-line when he sees a new patient with congenital heart disease," he says. "The goal is to increase the knowledge-base so adult patients get appropriate care, and are referred to a center in a timely fashion." With Dr. Welton Gersony, Alexander S. Nadas Professor of Pediatrics, Dr. Rosenbaum has coauthored the book "Congenital Heart Disease in the Adult," [December 2001, McGraw-Hill] which deals exclusively with managing the adult patient. The book clearly identifies specific problems in each type of adult congenital heart patient.

"We take this mission very seriously," says Dr. Rosenbaum. "Our center is a model for teaching congenital heart disease to our adult cardiology fellows. We are working on several research projects involving cardiac imaging and right ventricular function that we hope will impact positively on the management of these adults. With the large numbers of adults with congenital heart disease, these issues will only become more critical."

—Susan Conova