Transitioning Care for Patients with Congenital Heart Disease

November 2015

​​For the first time in history, the United States now has more adults than children with congenital heart disease. 

Congenital heart disease is a general term that covers a range of defects that involve abnormal or incomplete development of the heart. One in 100 children are born with a heart defect in the U.S. – 40,000 babies each year – but advances in surgical and perioperative care over the last 30 years mean that most patients, even those with very complex defects, are now living well into adulthood. 

Yet with such a boon comes significant challenges, not the least of which is the issue of transitioning this new population from pediatric care to an adult health setting.

Joseph Kay, MD​, associate professor of cardiology, runs the University of Colorado School of Medicine adult congenital heart disease program​, which spans both University of Colorado Hospital and Children’s Hospital Colorado. He is one of only three cardiologists in Colorado trained in adult congenital heart disease (ACHD). (Dr. Kay pictured, right)

“Lesions in the heart that were fatal in the early 1980s now have just a 10–15 percent mortality rate,” he explained. “It’s important to ensure continuity of care because although these patients may not experience issues in adolescence, as they age and the scars in their hearts evolve, more problems may develop in the third to fifth decade of life. We have to be able to catch these before they become more serious.” 

A Unique Disease 

Dr. Kay’s situation is unusual, because he works in such a specialized area of cardiology. He is trained in both pediatric and adult health care, and splits his time between Children’s Colorado and UCH. 

​“Although it is sometimes a challenge to work in both environments, you adapt and identify what you need in each area,” he said. 

“A large part of what I do relates to the transition of care, because as this population ages, many of them are lost to care. When they realize they need care again many think any cardiologist will do, but multiple studies show that this is not the case.”

Liz Yeung, MD,​ assistant professor and medical director of the ACHD transition program at Children’s Colorado, agrees that continuation of care is vital but says that her patients face several obstacles. (Dr. Yeung pictured, left)

“Trust is a major factor,” she said. “Many patients and their families have seen the same pediatric cardiologist since they were infants, so they don't really want to switch providers. Also, even with the affordable care act, finances are still a complicating factor for many people.” 

Growing to Meet the Need 

It is estimated that there are between 1.3 and 1.5 million ACHD survivors across the U.S., with a conservative estimate of 16,282 in Colorado. For Dr. Kay and his team, however, their reach extends much farther. 

“We currently serve roughly 2,500 patients, from northern Montana down to Texas and the Mexico border, and across to Kansas and Nebraska,” he said. “We know there are many more we need to reach, but as every patient’s needs are different and one approach does not fit all, we need more staff to cope with the demand.”

Depending of the complexity of the heart defect, ACHD patients need to be seen regularly, every six months to two years. In order to meet this goal, Dr. Kay is attempting to create a base at the Anschutz Medical Campus to act as a transition center. He has hired an ACHD transition coordinator, Molly Wallrich, BSN, RN, CPN, to help achieve this. Her job is to educate patients and their families, and prepare them for the transition. (Molly Wallrich pictured at right)

Preparing for the Transition 

“Typically I start seeing patients around age 12 to begin informing them about their specific defect and what the future may hold,” said Wallrich. 

“At around age 17 or 18, we transfer care to one of our three ACHD providers, who continue to see the patient at Children’s Colorado. When the patient is ready to complete their transition, usually around age 22, we transfer their care to UCH.” 

In addition to preparing patients for the move, Wallrich has several other duties: she teaches practical skills like how to order repeat prescriptions, administers transition-readiness surveys and offers ACHD-specific birth control advice. Since almost 50 percent of ACHD patients suffer from depression, she also performs a depression screening, in line with ACC and AHA guidelines.*

The Value of a Transition Program 

The staged approach of having patients see an ACHD specialist at Children’s Colorado before the physical transfer to UCH is an attempt to reduce the shock of a new hospital environment. 

“Some patients may be turned off to an adult health care setting,” said Dr. Kay. “Children’s hospitals tend to be more nurturing, with the provider taking on more responsibility for the child’s health. At an adult hospital, the onus is on the patient to take responsibility. In addition, since adult hospitals are larger, it is easier for this unique population to get lost in the sheer volume of patients.” 

Multiple studies have shown that a formal transition program can have a large impact on a patient’s attitude to care; without a transition program, almost 60 percent of ACHD patients are lost to care, while after the introduction of such a program the rate drops to about 10 percent.**

Empowered for the Future 

As the transition coordinator, Wallrich says that she feels fortunate to be involved in the project. “It’s a real privilege for me to help someone who comes in not knowing anything about how to manage their condition,” she said. “They leave feeling empowered and more prepared for the future.” 

However, she explains that some teenagers want to distance themselves from their health problems and lead a normal life. 

“It is hard to see people who don’t care for themselves or see their own potential. There are a couple of patients who keep me up at night,” she admits. “One is practically homeless. Another supports his mom and brother by working in construction, which is not an ideal profession when your condition limits how much weight you should lift.” 

“This young man has a lot of responsibility on his shoulders, and we can’t change that. What we can do is work closely with him to monitor his condition,” she said. 

“Unfortunately, the reality is that some of our patients can’t lead normal lives. But they can make their own normal, and I help them figure out how to do that.” 

Dr. Yeung acknowledges that having a dedicated transition nurse has been invaluable, but says there is still room for growth. 

“We really need to advance public awareness of this rapidly growing field,” she said. “Our patients often say they experience feelings of isolation, yet really this is the most common congenital defect.”

Footnotes:

  * Mussatto, K. A. et al. (2014) The importance of self-perceptions to psychosocial adjustment in adolescents with heart disease. J of Pediatric Health Care 28(3):251–261; Kovacs, A. H. et al. (2009) Depression and anxiety in adult congenital heart disease: predictors and prevalence. Int J Cardiol 137(2):158–64.

  ** Reid, G. J. et al. (2004) Prevalence and correlates of successful transfer from pediatric to adult health care among a cohort of young adults with complex congenital heart defects. Pediatrics Mar(113): e197–205; Mackie, A. S. et al. (2009) Children and adults with congenital heart disease lost to follow-up: who and when? Circulation 120(4): 302–9.