A New Center Treats Congenital Heart Defects

By Susanne Hartman
Penn Medicine - Fall 2005

Jim Hendrix wasn't your typical newborn or even 13-year-old, for that matter. “I was robbed of a normal childhood,” Hendrix explains. “I couldn't keep up with the other kids. I was always tired and short of breath and had to rest.” Hendrix, from Ocean City, NJ, was born with a defective heart.

Although doctors detected the defect before Hendrix's second birthday, when he “blacked out” during a tantrum, they did not perform surgery to fix it until he reached 13. Hendrix's disease was Tetralogy of Fallot, commonly called “blue baby” syndrome, a condition consisting of a number of different congenital defects within the heart.

In addition, the little boy's strained heart was tough on his entire family, emotionally and financially. When Hendrix caught a serious bacterial infection of his blood and spent his seventh birthday in the hospital, his parents were by his side while his grandmother cared for his three sisters.

“There was always a constant worry my heart would fail, due to the limited amount of ways to help children born with congenital heart defects back then,” says Hendrix. “I'm lucky that I survived long enough for technology to catch up and help me.”

Extraordinary advances in medicine have allowed many patients like Hendrix to live longer, yet in 2005, most primary-care physicians and even cardiologists still have not been trained to care for these complicated patients.

Jim Hendrix and Friend

Enter Gary Webb, MD. Regarded as one of the world's foremost authorities in adult congenital heart disease, he recently was recruited to lead the new Philadelphia Adult Congenital Heart Center. It is the first of its kind in the Mid-Atlantic region and one of only a few in North America. The joint venture combines the resources of both the University of Pennsylvania Health System and The Children's Hospital of Philadelphia, each nationally recognized for excellence in pediatric and adult cardiac care.

The center opened its doors this summer to help care for this small yet quickly growing group of cardiac patients. Many patients born with congenital heart defects had surgery in childhood and are now living well into adulthood. In fact, nearly 1 million adults are now living in the United States with congenital heart defects, including an estimated 40,000 such patients in the Greater Philadelphia region.

The Philadelphia Adult Congenital Heart Center has around 30 physicians and surgeons on the team from such areas as anesthesiology, cardiology, cardiovascular surgery, electrophysiology, cardiac catheterization, genetics, heart failure and transplantation, pulmonary hypertension, reproductive services, and imaging services. Desiree Fleck, CRNP, heads the nursing services. The center also has a research program.

The team will treat many adult congenital heart conditions, such as cyanotic defects, congenital valve defects, coarctation of the aorta, and septal defects, as well as adult congenital heart defect issues that affect pregnancy.

As leader of the center and professor of medicine, Webb brings a 30-year passion for this specialized area of medicine. Yet, back when his interest was first sparked, says Webb, “I was surprised to learn that the health-care systems didn't care very much about these patients. The progress in delivering care to these patients who need it was very slow and continues to be slow. I chose this area because there's not only a great need for people's involvement but to also make a significant contribution.”

Of the nearly one million American adults with congenital heart defects, half are listed in medium- and high-risk categories, which means they face premature death, serious complications, and a need for further treatment. But according to Webb, most adult cardiologists are not trained in the field and are not comfortable dealing with it. The result, he says, is a national shortage in skilled providers who work in this area.

Thus, as Webb sees it, the Philadelphia Adult Congenital Heart Center fills a void. It provides patients with access to a multidisciplinary team of experts in congenital heart defects who can provide the kind of care that they need. In addition, the center offers patients better information about their specific problem. With the sounder and more up-to-date information, patients are able to take greater charge of their own health.

Part of the Team at the Philadelphia Adult Congenital Heart Center.
From left to right: Gary Webb, MD, director; Desiree Fleck, CRNP;
Richard Donner, MD; and Martin St. John Sutton, MB, BS

Webb was recruited from Toronto, Canada, where he served for 18 years as director of the Toronto Congenital Cardiac Center for Adults and as professor of medicine at the University of Toronto. A fellow of the American College of Cardiology and of the Royal College of Physicians and Surgeons of Canada, he is founder of the International Society for Adult Congenital Cardiac Disease. He has some lofty goals for the new center here, hoping to eventually see 2,000 patients a year. As he puts it, “I've seen what excellence is in research and patient care and training. I want Philadelphia's program to emulate what we were able to accomplish in Toronto – and exceed it whenever possible.”

According to Webb, the more adult congenital heart disease patients the center sees, the more they will be able to help them. So the center's team is eager for referrals.

“Our goal is to help our patients live a normal life,” says Webb. “Patients who are at risk for further complications must get monitored regularly and avoid future problems rather than try to fix it after the damage is already done. We need a partnership between pediatric and adult care systems so that adults can get appropriate care in the course of their adult lives. They need to be educated and trained when going from childhood to adulthood to continue to monitor their own health so that their perhaps false feelings of good health don't ultimately cost them their future.”

That is precisely the trap Jim Hendrix almost fell into. After undergoing his first open heart surgery back in 1964, Hendrix regained his strength and finally got to live the life of a normal teenager. His doctors warned him to take it easy and get a desk job and play golf. Hendrix did the opposite – surfing, skydiving, and mountain climbing his way through life for the next 29 years. Says Hendrix, “I was out to prove I could do anything that anyone else could do.”

Along the way, Hendrix began by having a checkup every few years, but then less frequently. He felt so well that he didn't go see any doctors. A year ago, however, he started experiencing numbness and a tingling sensation in his hand. Also, he kept waking up with a numb leg. After enjoying decades of wonderful health, he knew something was wrong.

Hendrix checked into a local hospital to get tests done and was eventually referred to Webb at the Philadelphia Adult Congenital Heart Center for care. The 54-year-old man, who'd had his chest opened up and repaired already once in his life, went through the procedure again. Last May, at the Hospital of the University of Pennsylvania, two members of the center's multidisciplinary team performed the surgery. Alberto Pochettino, MD, a Penn cardiothoracic surgeon, and Thomas L. Spray, MD, chief of cardiothoracic surgery at Children's Hospital and the Alice Langdon Warner Professor of Surgery at the School of Medicine, closed a hole between the two upper chambers of Hendrix's heart, put in a pulmonary valve substitute, and stopped another valve from leaking. The surgery ultimately stopped the atrial fluttering of his heart.

In August, Hendrix celebrated both his 30th wedding anniversary to his wife, Barbara, and his 55th birthday. He is looking forward to many more years of riding his motorcycle.

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