Still another aspect of the program is helping adults live with the consequences of their heart disease and the treatment they received decades ago. "What the medical community did for pediatric patients in the past may turn out, in some instances, to be a mistake, but it was the best method of treatment available at that time," Dr. Eldredge stated. "For instance, years ago, surgeons used to routinely remove the pulmonic valve in patients with tetralogy of Fallot," explained Dr. Calderon. "It seemed like a good idea at the time, but how can someone live without a pulmonic valve? You can for a short time, but the expectation then was that these patients would not live long, healthy and productive lives. The expectations have changed because we're getting better at what we're doing and we're getting smarter with the data we collect," continued Dr. Calderon. "Today, one of the most common surgeries performed on adult patients who had a previous tetralogy of Fallot surgery is the replacement of the pulmonic valve."

According to Dr. Eldredge, an important purpose of the center is to look at the long-term results of living with congenital heart disease, the therapies used to correct them and the effects of the ordinary wear-and-tear that daily living has on individuals who had heart surgery 20, 30 or 40 years ago. "(We're) trying to define the lifetime expectancy for those who had procedure 'X.' We're early into that whole process," said Dr. Eldredge, adding, "They come here 20 or 30 years after having surgery as children and want to know what is going to happen to them when they're in their 50s and 60s; we haven't got a clue. We are trying to write the natural history so we have the answer." Dr. Eldredge notes that many patients are approaching the age in which other types of heart diseases like coronary artery disease, become issues. "We are counseling them regarding things like lifestyle changes for prevention and trying to find out what happens to a repaired tetralogy when the patient gets to be age 60 and has an infarct," Dr. Eldredge explained. "Getting them safely through other non-cardiac procedures, such as gall bladder surgery, also presents challenges," said Dr. Calderon.

"We have adult medicine and pediatrics working together, which is a huge advantage. Surgical cases are discussed at interdisciplinary surgical case conferences. We have anesthesiologists who are in tune to the particular needs of these patients, as well as a highly experienced nursing staff and social workers who are becoming more familiar with their needs," said Dr. Calderon. "There is familiarity with not just the lesions, but all the complications with which these patients present. Deborah is especially geared for this."

Another advantage Deborah has in offering its services to adults with congenital heart defects is a surgeon with extensive and rare experience in both pediatric and adult cardiothoracic surgery: Lynn B. McGrath, M.D., Chairman, Department of Surgery.

Drs. Eldredge and Calderon keep current in this new and growing field, reading the literature and getting involved in studies. "We are also very active in using the Internet and interacting with colleagues at other healthcare institutions," said Dr. Calderon. "I am frequently on the phone with other centers around the world discussing the best ways to manage patients." Deborah is currently involved with a cooperative, international, multicenter study on Fontan patients.

While UCLA has the oldest center for adults with congenital heart disease, in terms of size and experience, Deborah is ranked very high. Deborah currently follows between 2,500 and 2,800 adults with congenital heart disease, and the number is growing. Together, Drs. Eldredge and Calderon see between 80 and 100 outpatients each month. An average weekly congenital in-house caseload managed by Dr. Calderon typically runs between one to eight patients, including two or three cardiac catheterizations and one or two surgical patients. "We are among the top five in size, seeing about twice the number of patients as seen at the Cleveland Clinic," says Dr. Calderon, adding, "This is truly an emerging, growing field."

W. Jay Eldredge, MD. Director, Outpatient Services and Co-Director, Center for Adults with Congenital Heart Disease

Dawn Calderon, DO. Associate, Department of Cardiovascular Diseases and Co-Director, Center for Adults with Congenital Heart Disease

Early Detection Leads to Stroke Prevention

Of the half-million strokes that occur each year in the United States, 20 to 30 percent can be directly linked to carotid occlusive disease. According to Rodolfo C. Pascual, M.D., Chairman, Department of Vascular Surgery at Deborah, knowledge of current diagnostic techniques and proper management of patients with COD allow primary care physicians to help minimize the progressive deterioration of neurologic symptoms, prevent the onset of stroke and, most importantly, decrease the overall rate of stroke-related deaths. "Once a patient has a stroke, it is often too late to do anything for them because a complete occlusion cannot be corrected," said Dr. Pascual. "Therefore, early diagnosis and treatment of carotid artery occlusive disease is the key to stroke prevention.

By screening and treating COD patients earlier, a greater quality of life can be achieved."

Center Helps Adults with Congenital Heart Disease Navigate Medical Issues

Thanks to dramatic improvements in the early diagnosis and treatment of children born with heart defects over the last two decades, the population of adult survivors of congenital heart disease is increasing dramatically. In the last five to ten years, even those born with the most complex and severe malformations, such as hypoplastic left heart syndrome, are facing prospects for a far longer and more normal life than had ever been possible or even hoped for.

There are about 1 million people over the age of 18 in the United States with congenital heart disease, and this number increases annually by at least 30,000. As recently as 20 years ago, newborns with serious structural abnormalities of the heart were considered hopeless.

These patients who are now surviving into adulthood bring a whole new set of diagnostic and therapeutic challenges with them, not just to the adult cardiac community, but to the general medical community as well.

Calling on its vast, long-term experience in dealing with both pediatric and adult patients with congenital and acquired heart diseases, Deborah Heart and Lung Center recently announced the establishment of its Center for Adults with Congenital Heart Disease. It is one of only a handful of centers providing such services in the United States. The Director is W. Jay Eldredge, M.D., with Dawn M. Calderon, D.O., Co-Director. Dr. Eldredge has extensive experience in both pediatrics and diagnostic imaging, having served as Chairman of the Department of Pediatrics and as Chief of the Section of Cardiac Imaging at Deborah. He established Deborah's Adult Congenital Heart Disease Program in 1992. Dr. Calderon joined Deborah from the Cleveland Clinic in 1998 and is one of fewer than 50 cardiologists in the country with formal training in both adult and congenital cardiology.

The focus of the Center is to provide comprehensive diagnosis, treatment and counseling to individuals with congenital heart defects who are now facing special medical and health challenges in adulthood. The care of adults with congenital cardiac defects requires a level of expertise not widely available in most communities. "For over 40 years, Deborah has been a center of excellence in diagnosing and treating congenital and acquired heart disease in both adults and children," said Dr. Eldredge. "Our base of knowledge of congenital heart disease-its complexity, its anatomy, its diagnosis and treatment-is very deep and very extensive. Because of the vast number of these patients that we've seen here at Deborah, there's a huge foundation of experience and knowledge that you don't find in many other places," continued Dr. Eldredge. "And when you do find it, it is often in the setting of a children's hospital."

Deborah's team of medical professionals include experts in both pediatric and adult cardiology, congenital echocardiography, diagnostic and interventional cardiac catheterization, adult and pediatric cardiac surgery, pulmonology, anesthesiology, social work and a specially trained nursing staff. The medical staff offers in-depth understanding of the structural and hemodynamic abnormalities encountered in this group of patients, as well as knowledge of acquired heart diseases, internal medicine and catheter-based and surgical interventions. Both inpatient and outpatient services are available. "Patients in our program represent several subgroups," said Dr. Eldredge. "There are those former pediatric patients who did not need surgery, but who did require periodic follow-ups and are now adults. There are the pediatric patients who did have surgery, but are now coming back with problems and issues as adults. Finally, there is a third group who, surprisingly, have gone undetected and are just now being diagnosed with congenital conditions. This third group is much larger than I had expected. There is an element of diagnosis, there is an element of medical and surgical care, but there is also a huge element of counseling for lifestyles, occupational issues, pregnancy, expectations, and coronary artery disease prevention. We spend hours with patients on all those types of things, even genetics. While we're not geneticists, we are able to refer these patients to the right people," said Dr. Eldredge.

Indeed, many patients are now in their child-bearing years. For them, how congenital heart disease impacts their ability to have children-and the children they might bear-is a major concern. According to Dr. Calderon, the Center is well equipped to help and counsel pregnant women with congenital heart disease. "Two of the aspects that need to be addressed are women with congenital heart conditions whose pregnancy presents a potentially serious complication to them, and the genetic risk of potentially bearing a child with a congenital heart defect," said Dr. Calderon. "The latter also presents the opportunity for early detection of a congenital heart defect in the unborn child and to make arrangements for an intervention as early as possible."

A related issue is the concern women with congenital heart defects have about becoming pregnant in the first place. As Drs. Eldredge and Calderon point out, many women were told that it would be unsafe or impossible carry a baby to term. As it turns out, for many of those women, that is not true. "What has changed is both the body of knowledge and the expectation: many of these patients weren't realistically expected to live into an otherwise normal and healthy adulthood. True, there are those for whom pregnancy would still not be advisable, but at least now it's based on actual knowledge, rather than on some mythical 'oh, no, you can't do that!'" said Dr. Calderon, adding, "The most gratifying thing I have been able to do is clear someone to "'go make a baby.'"