FRIDAY, Aug. 17, 2018 (American Heart Association) — The growing population of adults with congenital heart disease could soon begin to see more personalized care, according to new guidelines released Thursday.

Diagnosis and follow-up care for people over 17 will be based on more than their heart anatomy. It will expand to include how the condition is playing out in their own body.

The guide from the American Heart Association and the American College of Cardiology was published in the journal Circulation and led by a group of 43 of the country’s leading doctors and researchers. It’s a wholesale revision of the first-ever U.S. guidelines on adult congenital heart disease issued 10 years ago.

A lot has changed in a decade.

“We’ve accumulated enough data to be able to personalize care for patients in a sense to more closely categorize them and make follow-up recommendations and decisions based on a new set of categories,” said Dr. Karen Stout, chair of the writing committee for the guidelines and director of the Adult Congenital Heart Disease Program at University of Washington Medical Center.

Other conditions, such as valve disease, an irregular heartbeat, or the outcome of surgery to fix a heart defect can influence the severity of a person’s congenital heart disease.

“We’ve learned there are a lot of other variables,” Stout said. “Just because someone has, for example, Tetralogy of Fallot or an atrial septal defect doesn’t mean they are the same as others with the same diagnoses.”

Congenital heart disease is a defect or abnormality that results when the heart or the blood vessels nearby don’t develop normally before birth. Most heart defects are spotted during early childhood, but sometimes aren’t discovered until adulthood.

And because of advances in surgery, medicine and research, more children are reaching adulthood with congenital heart disease. About 50 years ago, only a small number of patients with complex CHD survived to adulthood. About 90 percent do today. A recent study estimates more adults than children are living with congenital heart disease in the United States — 1.4 million adults compared with 1 million children.

The guidelines stress the importance of having a seamless transition of care from childhood to adulthood, so that children who grow up seeing pediatric cardiologists who specialize in congenital heart disease are then cared for by cardiologists specializing in adult disease. Stout said that will take coordination, an openness to cooperate and technology such as telehealth options.

“We are 30 to 40 years behind,” Daniels said. Pediatric cardiologists began certifying for congenital heart disease in the 1960s. “We have only 300 board-certified adult CHD cardiologists in the United States. We are on our way, but we need more training programs and we need additional [specialized] adult CHD centers throughout the country so patients have access to high-quality care. It’s something we are working to achieve.”

Both Daniels and Stout said they would like to see a registry of patients with congenital heart disease, similar to what’s in place in Europe and Canada, to better track long-term outcomes, conduct important research and ultimately improve patient care.

“What we don’t know is a great opportunity to do research differently than what we have done in the past,” Stout said. “We need a registry and to think differently on how we get real-world data.”

Registry discussions have been around for about a decade, Daniels said, but they take coordination and funding and a concerted effort by governing bodies to prioritize congenital heart disease patients. It would be one way to get at the raft of questions the new guidelines point out that are still unanswered in CHD research.

For example: How can networks of care be developed to ensure patients get the expert care needed, even with low numbers of specialists and centers? How can doctors ensure patients are not lost to care as they transition from pediatric to adult cardiology? How can doctors modify current congenital heart disease surgical procedures to prevent or reduce later development of heart failure or an irregular heartbeat?

“These aren’t minutiae questions,” Stout said. “They are big-picture questions that are surprisingly not known in 2018.”

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