In the following interview, Lucy Roche, MD, adult congenital heart disease (ACHD) fellowship director at the Peter Munk Cardiac Center in Toronto, Canada, and pioneer of the combined ACHD-heart failure (HF) clinic shares her perspectives regarding this subspecialty.

What motivated you to pursue this specific training?

Today, even those infants born with complex congenital heart disease (CHD) are now expected to survive to adult life. To uphold the legacy of this success and to stand by our patients as they age, attention needs to turn to minimizing late complications.

Unfortunately, patients with complex CHD still die much earlier than their counterparts in the general population. Far too many die in middle age, and the leading cause of death is heart failure.

When I was training, there were not many options for those patients in most places. So, although my background was in pediatric cardiology, I felt there was a real need to contribute to that gap.

What training pathway did you take?

I completed a five-year training program in pediatric cardiology at the Freeman Hospital, Newcastle upon Tyne, England, which had lots of exposure to pediatric HF, transplant, extracorporeal membrane oxygenation, ventricular assist device (VAD) and ACHD.

I then moved to Toronto and spent two years as clinical research fellow within their pediatric HF program, completing a research thesis about HF in tetralogy of Fallot. Finally, I spent about 15 months as an ACHD fellow at Toronto General Hospital.

Can you describe the need for a combined ACHD-HF clinic?

HF represents the main cause of death among ACHD patients and is one of the most common reasons for hospitalization. At the moment, neither general adult HF specialists, adult transplant specialists nor ACHD specialists are entirely comfortable with this population.

Patients are falling through the cracks. Our specialty (and more importantly, our patients) needs expert teams of care providers who can address this problem. An ACHD-HF clinic led by an ACHD cardiologist seems to be an excellent way of coordinating care and is a great entry point to help failing ACHD patients navigate the complex choices ahead of them.

Providing a home for this complex patient group also means we can consolidate our clinical experience, collect data to learn from this new patient cohort, and provide a great training opportunity to help more health care providers to grow confident in a new type of care.

What does the ACHD-HF clinic at Toronto comprise?

We started the clinic back in 2012, and initially it was just me. We take referrals from within our ACHD team, HF and transplant team, general and ACHD cardiologist outside of UHN (Toronto's University Health Network) across Ontario, and even from beyond. There are clear guidelines as to what type of ACHD patients the clinic can help.

Today, the ACHD-HF clinic is the access point to all advanced HF therapies for ACHD patients in Toronto. We involve colleagues from many other specialties in patient care including adult HF, congenital surgeons, hepatology, pulmonary hypertension, social workers, psychologists, palliative care and more to provide ACHD patients with the advanced and multidisciplinary care they need.

In 2018, we were delighted to have another ACHD-HF staff cardiologist join the team. We are working towards appointing our first dedicated ACHD-HF fellow in 2020. These are important steps to establish capacity for sustainable growth and program development that will allow us to grow the team into cohesive ACHD-HF program over the coming years and further promote a scholarly focus.

How are these patients optimized in the ACHD-HF clinic?

The care is highly individualized and complex, almost always involving multiple specialties. It is very important to discuss with the patient the type of care we can offer and explain how our team works during the early clinic visits.

By listening to the patient and their family, we find out what is most important to them and incorporate the patients' perspectives and values into the care plan. Once the diagnostic information is gathered, we discuss the potential management strategy.

We always start by considering if there are conventional (non-transplant) surgical or percutaneous interventional options, even if those options are quite high-risk.

We have to keep in mind that they will generally be better served by keeping their own heart for as much time; however, we do not want to jeopardize their chances of doing well post-transplant by waiting too long. It is a delicate balance that can be difficult to find.

What have been the overall outcomes of the combined ACHD-HF clinic?

Out of all of those referred to our clinic, the rate of death, transplant or VAD is approximately 30 percent, with death being the least frequent of those endpoints. Since we started the clinic in 2012, the number of ACHD transplant performed in Toronto has doubled, and the perioperative mortality after transplant has dropped markedly so that outcomes for ACHD transplant in Toronto are now very similar to those from non-ACHD transplant.

How do you integrate palliative care in ACHD-HF clinic?

Every single patient in the ACHD-HF clinic finds themselves in a difficult position, facing an uncertain future and making choices about management that usually come without guarantee of success. We talk about issues related to advanced care planning early in the process and continue as patients travel on their journey.

The style of communication and whether or not we use tools like advanced care workbooks or engage with psychologists or palliative care professionals very much depends on individual and family needs. Many of our patients have young children as well as elderly parents, and their focus of concern is often on how they support the people around them and what will happen to those people if they die or spend prolonged periods in hospital.

It is very important to address those fears and discuss practical steps that can help patients manage these issues during this phase of their life journey.

What is the biggest challenge you have faced during this endeavor?

The biggest challenge is the size and complexity of the ACHD-HF population. Currently, it is disproportionately larger than the number of providers and resources – and this picture is expected to worsen in future years.

Would you encourage quaternary centers in the U.S. to launch a combined ACHD-HF clinic?

It is highly dependent on each institution's volume, needs assessment and available expertise. Certainly, it would be beneficial for ACHD patients to have access to a regional ACHD-HF center of excellence.

For high-volume ACHD centers that are fairly robust in the clinical, surgical, imaging and electrophysiology aspects, and are collocated or have strong links to general adult HF and transplant centers, I would consider ACHD-HF to be the next step.

What advice would you give to our ACC FITs?

I would encourage them to pursue ACHD as a subspecialty and persevere despite the long years of training. This specialty is very rewarding in many aspects. The patients with ACHD have come a long way to this point. They have gone through multiple surgeries and procedures.

If we are able to help them accomplish their goals and live healthy adult lives, we not only benefiting them but are also protecting the efforts that every member of the pediatric health care team, patient and family has already put into their care.