Stanford Medicine Newsletter Updates For the Local Community

 

Matters of the heart

Program offers lifelong care for patients

Michael Murphy's heart surgery as a child put him at risk for unique health complications that are managed at Stanford's Adult Congenital Heart Program.

   

Michael Murphy's mother made special keepsake boxes for all seven of her children to preserve the typical memorabilia of childhood. In Murphy's box was a detailed record of the 1966 surgery that left a scar that runs 14 inches along his left side.

Murphy was just 4½ years old then, part of a special group of survivors: children born with heart defects that, just a few years earlier, had been untreatable. These children were once called blue babies — their skin colored by blood starved for oxygen by various structural defects. Murphy suffered from narrowing of the aorta, the main artery that sends oxygen-rich blood throughout the body. He visited a cardiologist every year as a child.

"I believed the pediatric surgery was a permanent fix," he said. "I didn't grow up thinking that it was something to be watched over for the rest of my life." Thirty years later, a cardiologist told him that the surgery, which kept him alive as a child, could put him at risk for health issues and might need to be repaired again.

"My reaction wasn't negative," Murphy said. "It was more like, 'I guess it's better for me to learn a little late that I need to see a new kind of adult congenital heart specialist than having my family learn about it after I had a major heart failure.'?"

Lifelong care

He is among the more than 1 million adult survivors of childhood heart defects who require care from a specialized group of cardiologists. Stanford's Adult Congenital Heart Program is one of the few centers with this expertise, providing services for adolescents and adults in one seamlessly coordinated package. The program is a collaboration between the adult hospital and Lucile Packard Children's Health Stanford.

Murphy, who lives in Southern California, came to Stanford's program for evaluation and treatment in May after his cardiologist noticed a change in his echocardiogram, a sign that his aorta may have narrowed again.

"Just because a surgery was successfully performed doesn't mean the fix is forever. Things can change. There is a definite need for lifelong care," said Ian Rogers, MD, clinical assistant professor of medicine at Stanford School of Medicine.

Using new catheter-based technology, Stanford doctors applied a balloon to expand the narrowed portion of Murphy's aorta. They also inserted a stent to strengthen the area — without the need for another surgery. But narrowing could happen again, Rogers said, so he will keep a close eye on Murphy.

New expertise

In addition to treating defects from birth, the program's cardiologists are trained to diagnose and treat problems that patients may acquire as adults, such as coronary artery disease. "When we saw that Mr. Murphy had plaque in his coronary arteries that could have easily led to a heart attack, we were able to harness our care team to address it," Rogers said.

It has taken decades — and a substantial number of children with repaired congenital heart defects to reach adulthood — for scientists to understand how treatment might affect good heart function. While research continues to answer such questions, doctors agree on the need for lifelong monitoring by experts at an adult congenital heart center.

However, a scarcity of trained practitioners has been a challenge. According to a 2011 study, there are only 100 adult congenital heart clinics in the United States, enough to care for only 5 percent of the affected population. Few fellowships in cardiology have traditionally included training in adult congenital cardiology.

Recent changes from the Accreditation Council for Graduate Medical Education and the American Board of Internal Medicine soon will establish officially recognized standards for such specialized training. Stanford is one of the few U.S. medical centers that offer specialized training in adult congenital heart disease.

New technology is making diagnosis and treatment for these heart patients more precise and far less invasive, Rogers said. Before advanced echocardiograms and other heart imaging methods became available, patients often would undergo a catheter-based exploration of the heart, which poses a greater risk. Improved imaging technologies also provide more detailed and functional information so surgeons "go into the operating room knowing so much more about what they will find — and that makes surgery safer and more efficient," Rogers said.

Growing need

Meanwhile, this special patient population continues to grow. About 40,000 babies are born each year in the United States with congenital heart defects. About 90 percent will reach adulthood, adding to a 5 percent annual growth of adult survivors. Researchers continue to collect data on how those defects, even once repaired, influence long-term heart and overall health. "Part of what we are doing with our program is to advance knowledge so people with congenital heart conditions can be treated more effectively," Rogers said.

Murphy said he feels doubly blessed. "I was fortunate to be born when my heart could be fixed well enough so I could grow up to run a few marathons — and live to be 52 in a time when medical science could fix me again," he said.

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