All Together Now? Exploring the Pros & Cons of CHD Survivorship Clinics

A small but growing number of survivorship clinics for congenital heart disease (CHD) patients are popping up in healthcare systems across the country, but some cardiologists wonder if the model is sustainable. 

After Jodi Lemacks’s son Joshua underwent three surgeries for a double-outlet right ventricle defect, his mother shuttled him to see a variety of specialists: a gastroenterologist, an endocrinologist, a physical therapist and, of course, a cardiologist and a pediatrician. “It was insane,” she says of her fragile child’s hectic healthcare schedule. “He was very high risk.” 

From the beginning of Joshua’s heart journey, Lemacks wished she could take him exclusively to the Children’s Hospital of Philadelphia (CHOP), where he had his surgeries, but it wasn’t feasible to trek more than 200 miles from their home in Richmond, Va., several times a year. “We just didn’t want to go anywhere else,” Lemacks says. “They knew his heart. They knew him.”

So, after CHOP launched a Single Ventricle Survivorship Program in 2011, Lemacks took Joshua, then 11, for a full day of across-the-board testing: blood work, a bone scan, an abdominal ultrasound, a liver function test and more. “It’s kind of a long day,” Lemacks says, “but it’s so much better than going to different buildings, different offices.” About a month later, after the clinic’s team of specialists had studied Joshua’s results, the Lemacks returned to CHOP to meet with the doctors and consider next steps. They learned that Joshua had liver disease and began a treatment plan. 

“The beauty of the survivorship clinic is they look at [the patient] as a general heart disease patient with these comorbidities, vs. a liver patient and gastroenterology patient,” says Lemacks, who is the program director of the patient advocacy group Mended Little Hearts. “They looked at [Joshua] as a person.”

Tale of two clinics

Adults with CHD now outnumber children with these conditions, and the need for long-term care for single-ventricle patients is greater than ever, says Kathryn Dodds, RN, MSN, CRNP, a clinical liaison nurse practitioner in CHOP’s cardiac center. Unlike some other CHD patients, single-ventricle survivors can be prone to life-threatening complications outside of cardiology. These complications can be difficult for cardiology experts to manage, Dodds says, especially without clear treatment protocols. Single-ventricle patients need a program that is “more than just cardiology,” she adds. 

CHOP’s solution is modeled on its cancer survivorship clinic. At first, the program focused on patients with complications, such as liver disease, but was soon expanded to include those without known complications. The survivorship clinic’s team of specialists includes two cardiologists, a hepatologist, an endocrinologist and an immunologist. The clinic isn’t meant to replace the care patients get from their primary cardiologists, Dodds says, but instead to identify underlying complications—like Joshua Lemacks’s liver disease—and help single-ventricle patients stay healthy.

CHOP’s clinic now has seen more than 400 patients. And while Dodds says it’s too soon to discuss outcomes, the clinic team published a study last year showing that a cohort of 67 predominantly asymptomatic children and adolescents with single ventricles all exhibited evidence for hepatic fibrosis, demonstrating the prevalence of the liver condition in Fontan survivors (J Am Heart Assoc 2017;6[5]:e004809). 

Dodds says she’s hoping to add a wellness component to the clinic that incorporates psychology and social work.

More than 800 miles away in Milwaukee, sits another example of a single-ventricle survivorship clinic, though one with distinct diff erences from CHOP. At the Children’s Hospital of Wisconsin, cardiologists studied 500 single-ventricle patients and found significant variability in choice and timing of tests, medication use and consultation with subspecialists on noncardiovascular problems, says cardiologist Salil Ginde, MD, MPH. The team developed a protocol to decrease variation and improve efficiency, but quickly began identifying complications in these patients that they hadn’t looked for previously. That led to the creation, about two years ago, of a multidisciplinary Fontan clinic to which single-ventricle patients are referred if the protocol uncovers an abnormality.

The clinic focuses on the Fontan population because the group faces so many non-cardiology issues, Ginde says. Patients with most other CHDs experience non-cardiology issues, but they’re not as universal as those faced by the Fontan population, though Ginde believes survivorship clinics might be of value for tetralogy of Fallot patients. “As cardiology providers,” Ginde explains, “we just don’t have the expertise and the training to manage liver disease and lung disease effectively.”

Based loosely on the classic tumor board, the Wisconsin clinic is composed of two cardiologists, including Ginde; two nurse practitioners; and one dedicated specialist each from hepatology, pulmonology, endocrinology and psychology who become experts in Fontan patients. The cardiologists and nurse practitioners triage clinic patients before meeting with the entire provider group to decide on next steps. Patients with active issues are sent to the clinic’s specialists. Now in its second year, the clinic has had about 40 referrals and works in conjunction with patients’ primary cardiologists.

The clinic was structured this way because making all of the specialists available for a same-day clinic proved too challenging, Ginde says, and there were issues with location, scheduling and long hours for patients. He acknowledges that the clinic is “very labor intensive, resource intensive,” and says it only works because of an invested team dedicated to the shared mission of improving long-term care.

Inherent challenges add up 

At Texas Children’s Hospital, adult CHD patients are mainly monitored from a cardiology perspective, says Peter Ermis, MD, a pediatric and adult cardiologist and associate director of the adult CHD program there. The hospital is in the process of developing a multidisciplinary program that focuses on single-ventricle patients, so Ermis is well versed on the inherent challenges.

A healthcare organization needs a critical mass of single-ventricle patients to make a survivorship clinic worthwhile, Ermis says. It also needs a core group of interested providers—cardiologists to spearhead the effort as well as at least a couple of hepatologists, hematologists and a social worker—all with wiggle room in their schedules. Other clinicians need to be willing to have their patients seen in such programs, and they need to be comfortable with the different opinions that could come with it. There must be buy-in from hospital administrators and leadership who are willing to undertake a process that is bound to have some growing pains. Figuring out sometimes complicated physical logistics, such as a centralized location for the clinic and a setup that differs from a traditional cardiac clinic, is key.

“There are a lot of logistics issues in starting something like this,” Ermis says, “and sometimes they just amount to too many issues.”

At Boston Children’s Hospital, there have been discussions about creating a Fontan clinic, but it hasn’t been put into place, according to Michelle Gurvitz, MD, MPH, attending physician in the cardiology department. There’s some discordance in the Fontan community, she says, between a clinic approach and development of a protocol. “If you know what testing somebody’s going to get, does that have to be isolated into a single clinic where people come for two days,” she says, “which is feasible for some people but completely not feasible for other people?”

The handful of Fontan clinics across the country all operate differently, with different groups of specialists and in different timeframes, Gurvitz says, referencing her own research and feedback she’s heard from others. Some Fontan patients might be hundreds of miles from a survivorship clinic but have a CHD clinic that’s much more accessible. The importance, Gurvitz says, is in establishing a protocol that’s the same for Fontan patients no matter where they’re seen. “As long as you have the components,” she says, “then you can get care to people.”