Coming of Age: Congenital Heart Disease Programs Hustle to Meet Needs of Adult Patients

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uw-heart.jpg - UW Medicine Regional Heart Center
Today there are more adults with congenital heart disease than children, and many of the adult patients require a lot of care, says Karen K. Stout, MD. Here Stout examines a patient at the UW Medicine Regional Heart Center.
Source: University of Washington Medical Center, UW Medicine Regional Heart Center.

The number of adults with congenital heart disease now exceeds pediatric patients who are born with the disease, thanks to continuing success in treatment and care through infancy, childhood and adolescence. But as lifespans expand, so do the pressures on healthcare systems and practices that for the first time are encountering adult congenital heart disease (ACHD) and the consequences of survival.  

The flex points

Between 1.3 million and 1.5 million American adults are living with CHD, according to estimates, and the rolls are expected to grow over time. That trend largely is due to improvements in survival. In one projection, researchers determined that the mortality rate for infants between 0 and 51 weeks with CHD had dropped 3.2-fold while the number of CHD patients between 20 and 64 years had increased by a factor of 2.3 over a 42-year period (Popul Health Metr, online Oct. 15, 2015). The study used positive responses to a question about CHD in the National Health Interview Survey, which likely is an underestimation of the actual prevalence.

“What we are seeing is that there are more adults [with CHD],” says Karen K. Stout, MD, director of the ACHD program at the University of Washington School of Medicine in Seattle and a co-author of the study. “Not only are there more adults, but in many cases they actually require a lot of care because the long-term sequelae of their repairs start catching up with them.”

The authors projected that the prevalence of ACHD would plateau around 2050, with about 2.31 ACHD cases per thousand adults in the United States. Other prevalence estimates nearly double that figure. Their study and previous analyses, while highlighting the victory achieved by pediatric cardiologists and surgeons, raise flags about the adequacy of resources in the adult setting.

“Everyone realized we were at this tipping point where we saw patients surviving well,” says Michael J. Landzberg, MD, director of the Boston Adult Congenital Heart program at Boston Children’s Hospital. “We recognized there was this tremendous burden of not just potential disease but real disease that was occurring: heart failure, arrhythmia, systemic vascular disease, peripheral disease. These are some of heaviest resource utilizers across the country and across the world.”

CHD, the most common birth defect in the United States, doesn’t fit easily into boxes. CHD refers to not one but a variety of conditions that can range in severity and complexity. It may be diagnosed during pregnancy, at birth or much later. The course of disease can vary greatly by individual patient, but early diagnosis improves the prognosis. Patients are rarely cured but rather have a lifelong disease that needs lifelong management.

“It is a strikingly heterogeneous group,” says Ami B. Bhatt, MD, director of the ACHD program at Massachusetts General Hospital in Boston. “People don’t necessarily have simple disease. They may have a disease that involves one lesion or multiple lesions and it may have been very complex when they were born or of minimal complexity, but none of that translates necessarily into the simplicity of their management.”

ACHD’s baby boomers

Stout, Landzberg and Bhatt are among ACHD physicians who are working to align existing and projected needs with a well-trained workforce, physician and patient access to ACHD expertise and a sound business model. A relatively new field, ACHD received its first guidelines in 2008 (J Am Coll Cardiol 2008;52[23]:e143-e263) and subspecialty certification in 2012. The American Board of Internal Medicine began offering additional certification in 2015. Landzberg contributed to the guidelines, an upcoming revision of them and the subspecialty certification effort, and Stout and Bhatt along with three other physicians co-wrote the American College of Cardiology core cardiovascular training statement for ACHD (J Am Coll Cardiol 2015:65[17]:1887-1898).

Bhatt also was the lead author on an American Heart Association statement on the care of ACHD patients who are 40 years old or older and aging-related issues. “That is our version of the baby boomer,” she says. “We included how to address congenital heart lesions which are first diagnosed in older age, consequences of childhood surgeries in adulthood as well as the importance of assessing risk for atherosclerotic disease which may develop independent of congenital heart disease.”

These also are the most cost-intensive age group. One study that assessed National