STANFORD, Calif. — Hand a napkin to Jim Wong, and he can
quickly diagram the four things that are wrong with his heart.
Between sips of coffee, he chats casually about how surgeons
implanted one artificial valve, repaired a second valve, sewed up a
small hole between the two upper heart chambers and did a maze
procedure to stop the arrhythmia
that hindered his heartbeat.
But Wong considers himself lucky. He found out before it was too
late that the heart problems he was born with hadn't ended, even after
he had childhood surgery to fix them, and now he lives near the
Congenital Cardiology Clinic at Stanford Hospital & Clinics.
Unlike Wong, most of the one million U.S. adults who have
life-threatening heart disease from their congenital heart conditions
are "lost," doctors say, because they still think the
surgery they had as children was all they needed.
"Some were told they were 'fixed,'" says Andrea Frederick,
RN, MSN, coordinator of Stanford's adult congenital cardiology clinic.
Now doctors realize their congenital defects weren't cured.
As doctors have recognized that these adults have lifelong problems,
they've started programs to help. Stanford has one of some 60 centers
nationwide dedicated to giving medical care to adults born with heart problems.
Wong, a 53-year-old Hitachi product strategist, already has had two
open-heart surgeries, and he'll need another operation when his
replacement pulmonary valve wears out. Fashioned from the pericardium
of a cow and a wire frame, it could last 10 years. Or maybe 15. Wong
has had his valve for six years now, "with no degradation."
Pause. "But you never know."
That's what it's like to live with adult
congenital heart disease. You're born with one or more defects
that can be repaired—but never permanently cured.
Decades ago, infants with holes in their hearts, or with missing
valves, vessels and chambers "basically used to die," said
Murphy, MD, professor of pediatric cardiology at Lucile Packard
Children's Hospital who also directs the adult congenital cardiology
clinic at Stanford Hospital & Clinics. "They either didn't
get surgery, or they didn't survive surgery."
Today, thanks to technological advances and an evolving
understanding of the body's critical pump, there is a new and growing
population of patients worldwide—survivors like Wong, who are living
into their 50s, 60s, even 70s.
Wong wears a medical alert medallion around his neck and carries a
health "passport" in his backpack that details his history
as someone who was born with Tetralogy of Fallot, one of more than 30
different congenital heart conditions. In an emergency, Wong's
passport tells responders to take him not to the nearest emergency
room but, if possible, to Stanford's emergency room.
There, specially trained cardiologists, electrophysiologists,
imagers and interventionalists can quickly diagnose and treat adult
congenital heart disease patients—by checking on a pacemaker,
or by doing an arterial switch procedure. Physicians at the clinic see
some 300 patients annually, and perform about 50 surgeries per year.
Some adults born with heart problems have stopped seeing a doctor
because they got discouraged when they saw physicians not familiar
with their conditions. "It was difficult to find someone who
understood their anatomy, and they just stopped going," said
Frederick, the nurse at the Stanford clinic.
Another high-risk subgroup of the "lost" are "young
adults and adolescents who think they're bullet-proof and don't need
health care," Murphy added. "And more than half of patients
between 20 and 30 have no health insurance."
Wong had his first open-heart surgery in 1960, at age five, and said
he was pronounced "fixed." He played sports in high school
and stopped seeing a cardiologist once he turned 16. In 2002, on a
business trip in Southeast Asia, Wong had to be hospitalized for seven
days with frightening atrial
fibrillation and atrial
flutter. "My heart would start racing, and wouldn't
stop," he recalls. "It was just horrible."
In Wong's case, the fibrillations, or rapid flutterings, were caused
by an irregular hole in the wall (septum) between the lower two
chambers of his heart. Because of the way his heart formed, he said
that the hole, or ventricle septal defect, "really messes
up" his circulation. When the rate of the flutterings, or
arrhythmias, are particularly fast, some heartbeats expel little or no
blood into the aorta, the body's biggest blood vessel.
He flew back to the Bay Area with a medical escort and contacted
Murphy, who'd been recommended by a U.S.-trained electrophysiologist
in Singapore. Tests indicated that he needed surgery, and Wong
returned to the Mayo Clinic where he'd had his first open-heart procedure.
Today, he sees Murphy for regular checkups—"And I've been
arrhythmia-free ever since."
Thanks to the Internet, Wong had been able to use the time when he
was hospitalized in Singapore to search the Internet for information
about adult congenital heart disease. "I found the Adult
Congenital Heart Association when I returned to the United States, and
the members on the message board made the whole traumatic event so
much easier to deal with." His work on behalf of the
organization has been a continual "giving back."
A past board chair of the association, Wong said that organization
is continually trying to identify the "lost," and encourage
them to get systematic, qualified care. "There are all those
people out there, and if they wait too long, they're going to die an
early death, or degenerate to the point where they may need heart transplants."
Murphy serves as a member of the medical advisory board of the
association, and he frequently is invited to talk with physicians'
groups about how hospitals and clinics can best care for adult
patients with congenital heart disease. At Stanford, ACHD patients are
transitioned from the children's program to the adult hospital at
about age 18, and Murphy argues that, "in the long run, adult
medicine needs to be the home for these folks."
In some ways, Wong and the emerging generations of congenital heart
patients are like survivors of childhood cancer. "They didn't
exist before, and their problems are unique and their diagnoses are
unique," Murphy said.