But first, cardiologists need to learn which patients to refer. To help them figure that out, Dr. Roberta Williams, chairman of pediatrics at Children's Hospital of Los Angeles, and Dr. Carol Warnes, professor of medicine at the Mayo Clinic in Rochester, Minn., are developing guidelines for cardiologists on how to manage adult congenital heart disease patients.
“It's sort of a cookbook, to give them a handle on what they should be looking for, what should trigger a referral to a specialized center, and what are the long-term outcomes,” Dr. Williams said. The guidelines, which Dr. Williams said would be available in about a year, are being cosponsored by the ACC and the American Heart Association.
In some geographic areas, “the best expertise [in this area] is in the children's hospital, because the anesthesiologists and the cardiologists there are best trained to manage these patients,” Dr. Mullen said.
Some cardiologists are starting to get the message. Dr. Murphy said his clinic sees about 10 patients a week, most of whom are complex and require long visits.
Dr. Joseph Perloff, founding director of the Adult Congenital Heart Disease Center at the University of California, Los Angeles, also has seen demand grow at his facility. “It was apparent decades ago that advances in surgical techniques in congenital heart disease would result in long-term survival, and there were no facilities or training programs” to help these patients, he said.
So Dr. Perloff started the center shortly after he arrived at UCLA in 1979; the center was initially open only 1 day a week. Today it is open 4 days a week and has a registry of almost 3,000 patients, most of them referred by cardiologists in the community. The Adult Congenital Heart Association, in conjunction with the International Society of Adult Congenital Heart Disease, is putting together a directory of self-described adult congenital heart clinics nationwide; so far, they have received 37 surveys from centers that describe themselves as such, according to Ms. Verstappen.
UCLA also has the first and largest training program for the specialty; the program admits one or two fellows per year, depending on the strength of the applicant pool, Dr. Perloff said. Physicians may apply for the fellowship after they finish residency, which must include 2 years in general cardiology and some pediatric cardiology as well. The fellowship lasts 1 year, “but usually the fellows stay on another year to complete a research study or develop a particular area of interest,” he noted.
Other training programs include those at the University of California, San Francisco, the Mayo Clinic, the University of Iowa, Iowa City, and the University of Pennsylvania, Philadelphia. But more need to be started, Dr. Perloff said.
He noted that currently there are only about 1,200 board-certified pediatric cardiologists, far too few to take care of the estimated 900,000 adults with congenital heart disease nationwide. In fact, “there are now more adults with congenital heart disease than infants and children,” he said.
One problem with recruiting people into training programs is that for cardiologists, “training is already very long” with 4 years of residency and 2 years of specialty training, Dr. Williams said. “To sustain this kind of [subspecialty] training, other aspects of training may need to be shortened, maybe by double-counting certain things, or having a loan forgiveness program or something to help them, because if you delay starting practice for 2 more years, the debt burden is so high a lot of them wouldn't be able to afford to do it.”
This situation is not helped by the fact that many adult congenital heart disease patients have trouble getting health insurance. Coverage for these patients under their parents' insurance policies typically stops when they turn 18 or 21. “How many 19-year-olds working at whatever job someone that age can have, will have health insurance?” Dr. Williams said. Treating uninsured or underinsured patients then adds to the physicians' financial woes.
To bring more attention to the issues surrounding these patients, the Adult Congenital Heart Association and several other organizations sponsored a “lobby day” in Washington on Feb. 8, aimed in part at getting Congress to fund a registry of congenital heart disease patients. “We need basic data to help us plan for the needs of adults with congenital heart disease; we need to do evidence-based medicine,” Ms. Verstappen said. “We also want to help children with congenital heart disease. Right now, we are doing surgeries in children without the data we could have from adults.”
