This post was authored by Keri Shafer, MD, Boston Children’s Hospital and Brigham and Women’s Hospital.

The care of adults with congenital heart disease (ACHD) is a rich and rewarding experience filled with unique challenges, some unexpected. I began ACHD fellowship enthusiastically determined to improve my understanding of complex cardiac physiology with questions swirling through my mind such as “What is anatomic malposition?” and “How is a Kawashima performed?” I soon learned that ACHD care is much more than that. Quality care requires a comprehensive understanding of the function of every organ system as years of congenital heart disease can take a toll on the lungs, kidneys, liver, etc.  Growing up with congenital heart disease can also affect patients’ approach to nearly every aspect of their lives. Therefore, the most successful ACHD physicians continually demonstrate compassion, patience and excellent communication skills when caring for their patients and families. Paramount among these skills is the ability to help patients and families through what can be the most difficult part of care: end of life.

The outcomes of patients with congenital heart disease are significantly better than prior decades: 90 percent of all babies born with congenital heart disease will reach adulthood. Age at death in congenital heart disease patients has been trending upwards, and recent data suggest that in tertiary referral centers outcomes are much better than previously seen. Yet while the overall trend is encouraging, survival of patients with complex disease is lagging behind. This final fact was underscored repeatedly throughout my fellowship training – patients with complex cardiac anatomy are often taken from us much sooner than we would like. By caring for these ACHD patients, I have come to realize that their end-of-life care can be particularly challenging – much more so than other patient populations I encountered during my internal medicine and general cardiology fellowship. In those parts of my training, I learned to intellectually tackle the challenges and address the poor outcomes inherent to the practice of medicine. I was also taught the fundamental concepts patients and families find important at the end-of-life including comfort-focused care, patient autonomy and continued compassionate communication. However, application of these important concepts can be difficult in the ACHD population for a variety of reasons inherent to their unique path through medical care. There are three topics that have consistently emerged for my patients and me:

1. Our poor prognostication: “But he was working full time yesterday?!”

Most ACHD patients live in a delicate balance, and predicting decline can be very difficult. As I have personally experienced many times, seemingly small issues can result in a patient’s rapid decline into extremis. We discover that our patients are often compensating for multiple issues that can quickly and simultaneously unravel with only a small insult. The slight rise in creatinine or barely perceptible increase in breathlessness may be the only sign of a profound change in intracardiac hemodynamics.  This subtlety translates into a much greater difficulty in predicting the timing of decline than in other patient populations.  As a result, we often lose precious time that could have been used to prepare patients and families for inevitable outcomes.

2. Families and ACHD patients have heard it before: “So THIS time he’s really sick?”

Many ACHD patients have amusing stories about the end-of-life discussions they’ve had in the past.  One of my favorites: “Remember my first cardiologist? He told my mother I’d die before I reached 10.  Now he’s dead and I’m still feeling fine… Ha!” While I find these stories inspiring, they can be impediments when the ACHD patient is actually at the end of his or her life. Usually, the patient has beaten the odds so many times before that it is difficult for him or her to believe that the expectations have truly changed. And because we struggle with prognostication, it’s very difficult to know how certain we can truly be that the patient will not recover.

3. My own (provider) biases: “But he’s my age.”

Few groups of early career physicians have to deal with the mortality of their peers; and I would venture to say that we all find it more difficult than expected. Despite the fact that I’d coped with the deaths of a diverse group of patients during my previous six years of post-graduate training, losing an adult with congenital heart disease feels different. One significant difference is that many ACHD patients could be (and in some cases are) my friends and colleagues.

As a new ACHD provider, I work to improve my clinical acumen in caring for these complex patients and endeavor to develop the skills of my colleagues for knowing when a minor change heralds significant abnormality. Simultaneously, we all look for new research avenues to better understand physiology in such a way that we might delay morbidity and mortality. As our newly recognized subspecialty grows, we must diligently work on improving and increasing the frequency of our discussions on advanced care planning. However, we must not forget the additional difficulties providers and trainees may face in caring for this unique population and work to improve education in this area.

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