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Finding 'lost' survivors of congenital heart defects

- By Diane Rogers

Credit: Norbert Von Der Groeben Jim Wong

Jim Wong was born with a heart condition that led to surgery at age 5. He thought the problem was fixed until he was hospitalized with arrhythmias some 40 years later.

Hand a napkin to Jim Wong, and he can quickly diagram four things 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. Now he lives near the Adult Congenital Cardiology Clinic at Stanford Hospital & Clinics.

Unlike Wong, most of the 1 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,'' said Andrea Frederick, RN, MSN, coordinator of the adult congenital cardiology clinic. Now doctors realize their congenital defects weren't cured.

As doctors recognized these adults have lifelong problems, they've started programs to help. Stanford has one of some 60 centers nationwide giving care to adults with congenital heart defects.

Wong, a 53-year-old Hitachi product strategist, already has had two open-heart surgeries, and he'll need another when his replacement pulmonary valve wears out. Fashioned from the pericardium of a cow and a wire frame, it could last 10 years. Or 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 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 and director of the adult congenital cardiology clinic. 'They either didn't get surgery, or they didn't survive surgery.'

Today, with technological advances and a better understanding of the heart, there is a growing population of patients - survivors like Wong - living into their 50s, 60s, even 70s.

Wong wears a medical alert medallion around his neck and carries a health 'passport' 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, 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 a pacemaker, or doing an arterial switch procedure. Physicians at the clinic see some 300 patients and perform about 50 surgeries per year.

Some adults born with heart problems have stopped seeing a doctor because they got discouraged when the doctors weren't familiar with their conditions. Another high-risk group of the 'lost' are teens '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 5, and 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 was hospitalized with frightening atrial fibrillation. 'My heart would start racing, and wouldn't stop,' he recalled. 'It was just horrible.'

The fibrillations, or rapid flutterings, were caused by an irregular hole in the wall between the lower two chambers of his heart. He said the hole, or ventricle septal defect, 'really messes up' his circulation. When the arrhythmias are fast, some heartbeats expel little blood to 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 an electrophysiologist in Singapore. Tests showed he needed surgery, and Wong returned to the Mayo Clinic where he'd had his first open-heart procedure. He sees Murphy for regular checkups - 'And I've been arrhythmia-free ever since,' he said.

Wong used the time while hospitalized in Singapore to search the Internet for information about his condition. He found the Adult Congenital Heart Association when he returned to the United States. 'The members on the message board made the whole traumatic event so much easier to deal with,' he said. He's worked for the organization ever since.

A past board chair of the association, Wong said it is trying to identify the 'lost,' and encourage them to get 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 on the association's advisory board and often talks to doctors' groups about best care for adults with congenital heart disease. 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.

Stanford Medicine integrates research, medical education and health care at its three institutions - Stanford School of Medicine, Stanford Health Care, and Stanford Children's Health. For more information, please visit the Office of Communications website at

2022 ISSUE 1

Understanding the world within us

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