Finding The "Lost" Survivors of Congenital Heart Defects:

Stanford Hospital & Clinics Program Provides Care to Adults Born with Heart Problems

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 Adult 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 Daniel 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.