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

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.”

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These also are the most cost-intensive age group. One study that assessed National Inpatient Sample data of patients with moderate to severe ACHD found that expenditures grew for all age groups from 2002 to 2013 but patients 44 years old and older posted the greatest spike (Circulation 2015;132:A18744). Reimbursement for the oldest group totaled more than $136 million compared with about $75 million for patients between 18 and 44 years old.

Landzberg estimates that between 100 and 150 centers offer specialized ACHD care—far too few, by his count, to meet the needs of 1.5 million adults, although accreditation processes underway may help grow those numbers. Between 500 and 1,000 physicians who identify themselves as having expertise in ACHD now seek certification, too, he calculates. Prevalence data support their decision, as does a study by Stout, Landzberg and others in the Alliance for Adult Research in Congenital Cardiology and the Adult Congenital Heart Association. They found that 42 percent of ACHD patients had a gap in care of three years or more, most commonly between 19 and 20 years of age, and 64 percent had moderate or severe CHD (J Am Coll Cardiol 2013:61:2180-2184).

The challenge is not only CHD’s heterogeneity but the variety of healthcare settings where a patient may be seen, Landzberg notes. He cites Provider Action for Treating Congenital Hearts, or PATCH, as one model that might improve access and quality of care. The Seattle system and Massachusetts General also use a hub-and-spoke model or telemedicine to ensure patients in rural areas many hundreds of miles away get access to state-of-the-art care.

PATCH is designed to foster partnerships between ACHD specialty centers and community cardiologists and internal medicine physicians who treat many ACHD patients. It provides educational opportunities, reassurance that the subspecialists are available when a patient needs more advanced care and messaging about recognizing when to use those resources. 

But PATCH cannot be universally applied, and it may be a tough sell to administrators, he acknowledges. Working collaboratively with a private practice requires a different approach from a hospital-based group or a university-based system and access to care varies by city and state. “We are still struggling with different models that make sense for every practice to engage in this educational process,” Landzberg says. The hub-and-spoke model and telemedicine also pose challenges. Building a robust program requires resources and a commitment from the hospital, Stout emphasizes, and infrastructural compatibility can pose problems.

They emphasize that achieving optimal health benefits the business, too. “Having better outcomes means better use of your resources and optimally the best advantage of the practice,” Landzberg says.

Resources & reimbursement

Being able to predict which patients will or will not require resources may improve efficiencies. An analysis by Bhatt and colleagues of high resource use by ACHD patients admitted to an adult hospital determined that the high resource admissions accounted for 32 percent of costs but only 10 percent of admissions (Congenit Heart Dis 2015;10:13–20). Their median cost was nearly four times higher with a median length of stay that was four times longer. Congestive heart failure, renal failure and complications were among the predictors of high resource use.

“Can we predict the people most likely to get in trouble? It is in everyone’s best interest to do so,” Bhatt says. “It will help those individuals in terms of their morbidity and potentially their mortality but it also will help the system” by allowing a more efficient allocation of resources.

While the medical community is beginning to recognize the benefits of ACHD subspecialty care, the insurance industry may be skeptical.

The Affordable Care Act has improved access to insurance to a population at risk of denial in the past, but without compelling evidence, insurers appear to be reluctant to pay for treatment and services outside their existing standards. ACHD physicians also aren’t always reimbursed for their time with remote consulting, Stout says.

“Insurers drive us bananas right now because they are so short-sighted,” she says. “They try to apply acquired heart disease norms and it doesn’t make any sense.” She recognizes that they lack the data to persuade payers otherwise, though. “It is up to us to prove that what we are doing is cost-effective care.”

Landzberg says they are developing quality metrics to be rolled out first in the ACHD centers and then applied to the larger community. Insurers will need to review and assess that process to determine reimbursement based on quality outcomes, eliminating what has been a thorn in the side if not a barrier to the field’s advancement. “Before something really takes a strong foothold among practitioners in this country, there has to be some payback for the practitioner in addition to the value of seeing your patient getting better,” he says.

In the meantime, well-designed programs that link ACHD expertise with other physicians who manage this growing patient population help achieve clinical and economic gains. “When you create structure, you find business success follows,” he says. “We are seeing that in our field. ... We are self-sustaining and in the black. That is not why we do it, but it is good to know that we are not loss leaders.”