Transitioning Care of the Adult Patient with Congenital Heart Disease

September 29, 2017 | Jordan D. Awerbach, MD, MPH

The majority of pediatric patients with congenital heart disease (CHD) fail to appropriately transition as adults to adult congenital heart disease (ACHD) specialists. Most experience a lapse in care, re-emerging later with significant illness under the care of providers with very limited CHD experience. In this article, I interview two ACHD providers and one ACHD patient about the process of transitioning patients with CHD.

Richard A. Krasuski, MD, FACC, is the Director of the Adult Congenital Heart Center at Duke University Medical Center. He completed residency in Internal Medicine at the Brigham and Women’s Hospital, following which he trained in adult cardiology at Duke. Dr. Krasuski recently returned to Duke, before which time he was Director of the ACHD program at the Cleveland Clinic from 2005 – 2015.

Wayne J. Franklin, MD, FACC, founded and directs the Texas Adult Congenital Heart Program at Baylor College of Medicine and Texas Children’s Hospital. He completed residency in combined Internal Medicine-Pediatrics at Duke University Medical Center, followed by a combined Pediatric and Adult Cardiology fellowship at Baylor College of Medicine and Texas Children’s Hospital.

Micheal Pernick, JD, was born with tetralogy of Fallot. He is a passionate advocate for adults with CHD and a member of the Board of Directors of the Adult Congenital Heart Association. Mr. Pernick is a graduate of Wesleyan University and NYU School of Law. He is currently an attorney at Paul, Weiss, Rifkind, Wharton and Garrison LLP, a New York law firm, where he practices in the litigation department and maintains an active pro bono voting rights practice.

When should we start the discussion with patients about transitioning care to an ACHD provider? What do you see as the important elements of successfully transitioning patients to ACHD care?

RK: In general, the earlier one can plant the seed about transition, the better. The guidelines emphasize doing this as soon as the congenital heart patient enters their teenage years. I would emphasize that this should not be a “one size, fits all” approach. There are patients with complex diseases and associated neurocognitive issues that may benefit from longer follow-up under primarily pediatric care. There are also patients with multi-organ system issues that may benefit from transition to ACHD care at an earlier time. Either way, bringing this up during office visits early is important – the patient should never feel like they are being abandoned and, likewise, the physicians involved need to communicate with one another to understand the special needs and nuances of each patient. Both sides (pediatric and ACHD) need to feel that this is truly a transition process and not just a change in providers. The most successful transitions, I have found, occur when there is open communication between physicians and advanced practice providers from both sides, and when there is a period of overlap when both sides are involved in the care of the patient. Patients need to understand this is a “team” approach and that everyone has their best interests at heart.   

WF: We have a formal transition process that begins at age 14. This is a coordinated effort between both the pediatric cardiologist and the receiving ACHD team. A vital component to our success is having a dedicated “transition team,” consisting of a pediatric cardiology nurse and social worker. They will initially meet with the patient and his/her family to begin the process, introduce the concept of care transition and evaluate the patient’s readiness for transitioning to adult care. After the initial visit, our team re-evaluates the patient annually and provides education along the way about the steps necessary to successfully continue their care as an adult. Once a patient has met the transition readiness criteria, they will have their first official ACHD clinic visit. There is no set age for the transition and it is heavily individualized to the patient. The transition team stays involved and continues to follow-up with the patient to ensure the process worked and resulted in a sustainable relation between the patient and their new ACHD provider.

MP: I transitioned to an ACHD provider when I was 22 years old. It was not a planned transition. I had just moved to a new city and was still followed by my pediatric cardiologist back home, but days after I moved, I contracted endocarditis. Everything worked out well – we caught the infection early and I had a long but successful recovery. If it had not been for that scary situation, I may not have transitioned. Now I have been seeing an ACHD doctor for years. I know this is the right person for me – my doctor and I have honest conversations about growing older with CHD, risks of acquired heart disease and how that interacts with my CHD, and other long term planning for a healthy life. This sort of medical care can only be provided by someone who specializes in treating adults with CHD.

Are ACHD patients in your institution primarily cared for in an adult or pediatric hospital? What do you see that has worked well in that model, and what obstacles have you had to navigate?

RK: We are extremely fortunate at Duke that we are essentially one big medical center on a single campus. We share facilities and have offices and labs that are close to one another. For example, while we have separate adult and pediatric catheterization laboratories, in essence we are about 40 yards from each other. We share storage space for equipment and we frequently work together. Our ACHD patients are admitted to a single inpatient service, with an attending physician that has training and experience in ACHD care. On the rare occasion when the attending physician has less experience with managing a particular disorder, we have ACHD consultation available. Being on the same floor (usually) in a single hospital greatly facilitates consultation care, particularly when advanced services (EP, mechanical support, etc.) are necessary. However, there are always obstacles to any type of model. The most challenging is establishing who should be called (pediatric or adult specialist) with particular circumstances – the best example is when there are EP issues. We have such phenomenal pediatric and adult EP specialists, with such depth and breadth of knowledge and experience, that it can be difficult to know who could best serve the patient. For instance, a patient with an interatrial reentry tachycardia usually falls under the domain of our pediatric colleagues, while atrial fibrillation, particularly when discussing potential ablation therapy, is best served by our adult EP group. Fortunately, the two sides have a terrific working relationship and often work together on complex cases.   

WF: Our ACHD patients are cared for at both our pediatric and adult hospitals – Texas Children’s and CHI St. Luke’s, respectively. They adjoin each other, which helps significantly when care needs to cross over from one facility to the other; for example, when an adult subspecialist is better suited to consult on an ACHD patient at the children’s hospital. The ACHD patient faces unique challenges at either facility. The pediatric hospital typically has a longer history with the patient, and caregivers tend to have a better understanding of CHD and the nuances of caring for specific congenital lesions. At the same time, they are often not equipped to tackle more complex adult medical problems (pregnancy, substance abuse, coronary artery disease, etc.). Many pediatric caregivers also express a discomfort or dislike of caring for the adult patient. This can hinder the quality of care that the patient receives. Lastly, medication errors are a big concern, as patients may be on unfamiliar drugs and providers are used to prescribing weight-based pediatric doses. In contrast, the adult hospital is well equipped to take care of the typical adult medical problems, but are often unfamiliar with patient’s anatomy or the operations they have had. Patients who are used to being taken care of at a pediatric facility may be unprepared for the “adult style” of inpatient care. Adult facilities also often do not have the same administrative interest or resources dedicated to ACHD patients as they do for acquired heart disease.

MP: My clinic serves both adults and children. If possible, it is important to go someplace where they see a lot of adults – everyone from the echo techs to the nurses spend much of their time working with ACHD patients. It is helpful to not only have a doctor but an entire clinic that is used to treating people like me.

Congenital heart disease tends to make the non-congenital cardiologist uncomfortable. What advice do you want healthcare professionals to know about caring for the ACHD population? 

RK: Do not be afraid to utilize the ACHD specialists that are around you. Our numbers are growing and our centers are increasing! ACHD patients can be extremely complicated (even though they may not look like it on the surface) and often downplay their symptoms or problems. I am amazed how often the patient tells me they are doing “great,” when objective evidence (echo, stress test, etc.) tells me differently. I always emphasize to my referring physicians that ACHD care is a team sport. The community provider (cardiologist, internist, family physician, advanced practice provider) is probably the most important member. As specialists, we are ready to get as involved as necessary and as much as the referring physician feels comfortable with. I often prefer to follow patients in concert with their primary provider, with the latter being the first point of contact. That changes, however, with the complexity of the patient and the comfort level of the provider. We have one most important goal in mind – the health and well-being of our ACHD population!

WF:  Use your ACHD team! We have a big interest in these patients, have resources dedicated to them, and have relationships with both the pediatric and adult teams who are involved in their care. The most important thing is that everyone works as a team.

MP: I have two main pieces of advice. First, make sure your patients are seeing the right doctor: an adult CHD provider. Non-congenital cardiologists are not qualified to treat patients with CHD, and pediatric cardiologists are not qualified to treat adults with CHD. This is not just my opinion: It is the collective view of the Adult Congenital Heart Association, American College of Cardiology and ACHD doctors across the country. Right now, only 10 percent of adults living with CHD are seeing the right doctor – and everyone else is at risk. As a non-CHD provider, you are best positioned to fix this significant public health risk by making sure your patients are seeing the right specialist. Second, make sure you are communicating with your patients’ ACHD doctors. When I see my general practitioner, it gives me tremendous comfort to know that he receives my files from my ACHD doctor and is familiar with the tests that are being run. I do not expect him to be an expert on my CHD care, but I do expect him to have a sense of what is going on, the work that is happening and how that might implicate the rest of my health.

This article was authored by Jordan D. Awerbach, MD, MPH, Thomas Anstrom ACHD Fellow in Training (FIT) at Duke University in Durham, NC.