Sang Hee Yoon (center) received care through the Adult Congenital Heart Program at Stanford. Also pictured are his wife, Min Wha Yoon, and (from left) doctors George Lui, Daniel Murphy and Katsuhide Maeda.
Norbert von der Groeben
At 61 years old, Sang Hee Yoon seems an unlikely patient for a children’s hospital.
Yet earlier this year, the San Jose, California, man was wheeled into an operating room at Lucile Packard Children’s Hospital Stanford as one member of a fast-growing group of adults whose congenital heart defects require the expertise of pediatric heart surgeons.
When Yoon was born, few babies with severe congenital heart disease survived childhood. Today, after a half-century of improvements in the ability to repair such defects, more than 1 million U.S. adults are living with congenital heart disease, and their ranks are growing by 20,000 per year. The triumph of their long survival brings new challenges for them and their doctors.
“Patients come back at 40 or 50 years old, telling us, ‘My doctor said I was cured,’” said George Lui, MD, medical director of the Adult Congenital Heart Program at Stanford, a collaboration between the Heart Center at Lucile Packard Children’s Hospital and Stanford Health Care. Some patients’ childhood surgical repairs were initially judged so successful that they never expected to return to a cardiologist, said Lui, who is also clinical assistant professor of cardiovascular medicine and of pediatrics at the School of Medicine. In other cases, the first surgery was so unusual and risky that the surgeon discouraged the patient from undergoing further operations.
But most adults with repaired congenital heart defects are not cured, doctors have learned. As their discipline has matured, cardiologists have honed their understanding of how to help patients like Yoon navigate the risks of living with lingering heart problems, as well as learned how congenital defects interact with cardiovascular problems people acquire with age.
The doctors draw on knowledge from both pediatric and adult cardiology to diagnose the current condition of patients’ hearts and provide lifestyle counseling, medical management and interventional and surgical treatments. They also work to help adolescents with congenital heart defects make a smooth transition from pediatric to adult care.
Surgery for tetralogy of Fallot
Yoon was diagnosed at age 21 in his native South Korea, when a test showed tetralogy of Fallot, a heart and blood-vessel defect characterized by poor blood flow to the lungs. The diagnosis explained his years of intense headaches, chest tightness, blue lips and nail beds, and severe shortness of breath. As a child and teenager, he could not walk more than a few feet without having to stop and squat to catch his breath. His body wasn’t getting enough oxygen. He also struggled with depression, feeling that he was too ill to lead a normal life.
“I couldn’t dream of marriage because with that condition you are not supposed to marry,” Yoon said, speaking through an interpreter about his memories of the time before his diagnosis.
I couldn’t dream of marriage because with that condition you are not supposed to marry.
Yoon’s November 1975 surgery was one of the first performed for tetralogy of Fallot in South Korea. His surgeon was unsure how well the repair would work, warning that Yoon would still have a heart murmur from a malfunctioning valve. But Yoon recovered strongly from the surgery and felt much better. He married, became a pastor, and he and his wife had four children. In 1999, the family moved to California.
Then, in 2002, Yoon was trimming trees at home in San Jose when he felt dizzy and faint. Some of his earlier symptoms had never completely vanished — he had lingering chest pain and trouble breathing at high altitudes. However, at checkups with cardiologist Daniel Murphy, MD, at Stanford’s adult congenital heart clinic, Yoon was initially reassured to learn his heart was in relatively good shape.
But eventually, things changed. Earlier this year, an MRI scan revealed that the right side of his heart had enlarged, a precursor to heart failure. As is common in adults with congenital heart disease, the malfunctioning valve was taking a toll. Blood was backwashing into his heart and making it work harder.
“Mr. Yoon’s right ventricle, the heart chamber that pumps blood to the lungs, had gotten bigger and bigger,” said Katsuhide Maeda, MD, pediatric cardiothoracic surgeon at Packard Children’s, who is also part of Stanford’s adult congenital heart disease team. “It was almost double its normal size.”
‘The boat is sinking’
Murphy and Maeda recommended a new heart valve, which would require open-heart surgery. The prospect made Yoon nervous, but the doctors reassured him that such procedures had become safer since his first operation in Korea decades ago, and cautioned that, without the valve, his heart would continue to weaken.
“Dr. Murphy’s explanation of his condition was perfect,” said Yoon’s wife, Min Wha Yoon. “He said, ‘The boat is sinking and water is coming inside the boat. If you have strong muscles, you can pour out water strongly, but if you do not have much strength, you can’t.’”
“If you wait too long, the heart doesn’t recover,” said Murphy, who is also professor of pediatrics at the School of Medicine. “We really try to protect the heart muscle by replacing the valve.”
That advice made up the Yoons’ minds. “After that explanation we decided: This is the time to have surgery again,” Min Wha Yoon said.
We don’t want them to be heart patients; we want them to be people who come here to get their hearts checked.
The surgery, on May 13, took about four hours. “We put in a bio-prosthetic valve made of pig tissue,” said Maeda, who is also clinical assistant professor of cardiothoracic surgery at the School of Medicine. “It’s a pretty standard procedure and it went very, very smoothly.” Sang Hee Yoon was definitely the only 61-year-old receiving surgery at the children’s hospital that day.
After he woke from the operation, he was surprised to find that the lingering pain he had endured for decades was gone. As he recovered, he said he felt healthier than he had at any other point in his life.
“Breathing is much, much easier than before,” he said. His generalized body aches and chest tightness are completely gone, too. His children and his 10 grandkids are delighted in the changes. “They are so happy about my condition,” he said. “Not only family members but everybody I know is saying, ‘You look so healthy!’”
The Yoons have already visited Kings Canyon National Park, a destination they chose for its mountainous scenery. “I feel such gratitude that now I can enjoy my new life,” Yoon said.
He’ll still need checkups, but Yoon is in better shape than many adults with congenital heart disease, half of whom are estimated not to be receiving any specialized care. Instead of facing their uncertain fate, his surgery has moved him into the category of healthy individuals the Adult Congenital Heart Program aims to create.
“We don’t want them to be heart patients; we want them to be people who come here to get their hearts checked,” Murphy said. “It’s about giving people healthier, happy lives by offering a lifetime of high-quality care.”
Stanford Medicine integrates research, medical education and health care at its three institutions – Stanford University School of Medicine, Stanford Health Care (formerly Stanford Hospital & Clinics), and Lucile Packard Children’s Hospital Stanford. For more information, please visit the Office of Communication & Public Affairs site at http://mednews.stanford.edu.