Laura Goldenschue takes antibiotics before going to the dentist, even just for a routine cleaning. The 59-year-old from Texas assembles a team of specialists when facing a procedure as minor as a cyst removal. She carries a tiny “health passport” that lists her doctors and explains she is an adult survivor of congenital heart disease.

But sometimes, all that doesn’t help. Emergencies happen.

In Wyoming several years ago, Goldenschue had shortness of breath, sweating and severe abdominal pain on her left side. It was a terrifying episode, though not unexpected for someone with congenital heart disease. But in Cody, an emergency room doctor insisted on airlifting her to the nearest major hospital, which had no specialists in adult congenital heart disease. Another hospital, only a couple hundred miles further, had an adult congenital heart disease program and was far better equipped to understand and deal with likely complications.

“That’s what’s hard sometimes, when doctors just don’t know what to do,” said Goldenschue.

A new report from the American Heart Association promises to help. It details the major organs — the kidneys, lungs and liver, for example — and other systems affected by congenital heart disease and describes evidence-based treatment options.

George Lui, M.D., is lead author the new scientific statement published in Circulation and said he proposed writing it partly because of incredible advances in cardiac surgical techniques. Today, there are more adult survivors of congenital heart disease than children with the disease, he said. A recent assessment estimates about 1.4 million adults and 1 million children in the United States are living with CHD.

“So we’ve been successful, and people with congenital heart disease are living longer, fuller lives,” said Lui, medical director of the Adult Congenital Heart Program at Stanford University, a Lucile Packard Children’s Hospital and Stanford Health Care collaboration.

But those patients have ongoing health issues that derive from their heart defects and from treatments over the years. Surgery to reroute major blood vessels surely saved the lives of many cyanotic or “blue” babies, but the rearranged anatomy and physiology also left them vulnerable to kidney and other non-cardiac diseases later in life.

Moreover, like everyone else, CHD patients get colds and need cavities filled, but they are often far more vulnerable to complications than the average person.

“The risk of infection persists lifelong,” the report notes, “with even small unoperated ventricular septal defects having a risk of infective endocarditis that is 20 to 30 times that of the general population.”

Goldenschue was born with four heart defects that threatened her life because her heart couldn’t deliver enough oxygenated blood to her body or deoxygenated blood to her lungs. She had a shunt procedure before she turned 1, and more corrective surgery at age 9. Her heart remains imperfect, but Goldenschue stays active.

“I can’t hike a mountain, but I can ride a motorcycle,” she said.

Texan Laura Goldenschue is among the 1.4 million U.S. adults with congenital heart disease. (Photo courtesy of Laura Goldenschue)

Texan Laura Goldenschue is among the 1.4 million U.S. adults with congenital heart disease. (Photo courtesy of Laura Goldenschue)

The new AHA statement on non-cardiac complications deals with common, broadly understood impacts of congenital heart disease — the lung troubles those patients often deal with as they age, common blood abnormalities in cyanotic patients, and the importance of regularly assessing for kidney dysfunction.

It also details other issues that are just now being recognized as important. For example, it’s only recently that care providers have realized the extent to which CHD patients are at risk for liver disease, endocrine abnormalities, atherosclerotic cardiovascular disease and cancer, Lui said.

“Non-cardiac complications in adult congenital heart disease patients may have an impact on long-term outcomes,” he said. “Should we be screening these patients for atherosclerotic cardiovascular disease or cirrhosis? I can tell you right now we don’t in every patient. We need more research in what modifiable factors can be targeted for prevention.”

Anitha John, M.D., Ph.D., director of the Washington Adult Congenital Heart Program at Children’s National Health System in Washington, D.C., agreed. John, who was not involved in writing the statement, called it “incredibly comprehensive. It does a fantastic job touching on a lot of the different, non-cardiac issues as well as critical research needs.”

“This field is in evolution,” John said. “Because patients are surviving, they’re writing their own natural history.” The new statement, she said, is a powerful acknowledgement of the challenges that these adult patients deal with for decades, and it is a practical guide for care providers.

John said she found especially compelling the document’s call for better research into the neurodevelopmental and cognitive impacts of congenital heart disease. “We are learning that different treatments and behavioral interventions can be instituted earlier to help patients better cope with illness-related depression, anxiety and learning challenges in certain areas,” she said.

A selection of non-heart complications in adults with congenital heart disease. (Credit: Circulation)

A selection of non-heart complications in adults with congenital heart disease. (Credit: Circulation)

When Goldenschue dealt with a dangerous endocrine tumor a couple years ago, she accidently learned — again — a lesson that Lui called one of the most important in the new statement: Get help from experts.

Goldenschue had tried to get the tumor, which was wrapped around a major blood vessel, treated near home. She eventually called experts at the Boston Adult Congenital Heart program, who suggested she send her charts and fly out.

“They had a whole team of doctors for me to consult with and they took care of it,” Goldenschue said. “But I had to have a special everything: cardiologist, endocrinologist, vascular surgeon. Even the guy who was the anesthesiologist knew about congenital heart defects.”

Lui and John said a key factor in the health and well-being of adult congenital heart disease patients is access to quality care, with physicians who specialize in their disease.

Goldenschue said that for her, the person who fills that role and has likely saved her life is her adult congenital heart doctor, also a pediatric cardiologist. “I wouldn’t be here today [without him],” she said.

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