A View From the End

What the Last Year of Life Can Teach us About Palliative Care on the Adult Congenital Heart Disease Journey

Jill M. Steiner: James N. Kirkpatrick


Eur Heart J. 2022;43(42):4493-4495. 

Graphical Abstract

Palliative care needs in adult congenital heart disease and areas for future research.

Thinking about death is not easy. Most medical care is intended to prevent, or at least ward off, death and the potential for suffering as a result of severe illness. Even when death is imminent, conversations about how to anticipate and manage uncertainty, fear, and grief are rare.

Despite impressive advances in care for patients with adult congenital heart disease (ACHD), progressive disease and premature death are unshakable realities. Many patients with ACHD are young and vibrant, certainly compared with many patients with acquired heart disease. They often face difficult medical decisions in earlier phases of life and, as a result, they may have different concerns, values, and perspectives from someone diagnosed with a serious illness after living a long, healthy life, or an older person struggling with multiple chronic conditions for years. Retrospective studies of causes of death and comorbidity in ACHD exist, but much less is known about the months to years leading up to the end of life. In addition, all of this presents a shifting target as patients in younger cohorts are living longer and may be less affected day-to-day by their congenital heart disease (CHD).

We have only recently begun to explore patients' experiences at the end of life and their views on topics such as palliative care and advance care planning.[1–3] It is likely that we can—and need to—improve the care provided to patients with ACHD approaching death. There is no accepted standard of care for end-of-life in ACHD, with guidelines recommending only individualized decision-making,[4,5] and care decisions are often a result of institutional experience and preference.

We applaud Van Bulck and colleagues for their important contribution to this nascent area of study. In this issue of the European Heart Journal, they present population-level data, rare in ACHD research, to report on the causes of death and patterns of healthcare utilization for patients with ACHD in the last year of life.[6] They found that just under half died of a cardiovascular cause and, of those, CHD was implicated in 30% of deaths. There was also very high resource use—87% of patients were hospitalized at least once in the last year, and 78% had an Emergency Department visit. Utilization increased sharply in the last month of life, with 70% hospitalized and half with an Emergency Department visit. Yet, despite these numbers, only 28% of patients saw their CHD physician in the last year and 11% in the last month of life. Together, these results suggest the need for multidisciplinary care and a strong network of resources (both inpatient and outpatient) to support patients in this last phase of life. This includes a critical need for mental health resources, social work and community organizations, and insurance structures that support comprehensive, consistent multidisciplinary care.

It should be recognized that CHD was listed as the cause of death for the minority of patients. With regards to advances in CHD care, this may be celebrated as a triumph—but it is also a reminder to consider the development of coronary and other cardiovascular diseases, as well as the many non-cardiovascular conditions that aging patients face.[7] Given the development of comorbidities as our patients age, ACHD clinicians cannot operate in a vacuum. We must foster relationships with clinicians in other specialties to co-manage beyond CHD and, in fact, beyond heart care, recognizing that CHD, no matter how complex it may be, is often only one piece of the puzzle.

A complicating factor is that there are not enough ACHD clinicians to serve this rapidly growing population. ACHD specialists care for only a minority of patients with ACHD, which may be what is reflected in the reported low percentage of ACHD clinician visits near the end of life. Access to ACHD care is a huge concern, especially for patients living in rural areas or from underserved backgrounds. We must therefore continue to work toward increased capacity,[8] through such avenues as expanded training, institutional partnerships, and telehealth, and with a focus on healthcare equity, to ensure that patients' care needs are met.

This also means asking our patients about their needs, rather than making assumptions, while ensuring that we conduct assessments of patients' emotional needs in addition to physical concerns. Mental health is rarely addressed by ACHD clinicians, despite the well-documented high prevalence of anxiety, depression, and post-traumatic stress[9,10] in this population. These can contribute to poor quality of life and are increasingly important in times of worsening health and at the end of life. The high proportion of patients who died by suicide in this study is alarming and should not go unnoticed. Ensuring patients have the necessary skills and resources to manage stress should be a care priority, operationalizing concepts such as resilience,[11,12] self-efficacy, and positive coping.

Something we cannot know from these data is whether this type or level of healthcare utilization was wanted by the patients who experienced it. A prominent concern in palliative care research is that intensive life-prolonging care may not be wanted by patients approaching the end of life, and that such care may be more burdensome than beneficial.[13] It is therefore important to elicit patients' goals of care, initially and at multiple points along their healthcare journey. Preferences expressed when in good health may change as patients' health declines, as it is hard to conceptualize such a time until it happens. That patients with ACHD may be living a decade longer than previously reported may also imply changes to patients' values, preferences, or palliative care needs. Of course, it is possible that such high resource utilization is entirely appropriate and does reflect ACHD patients' care preferences—perhaps having lived fewer years means patients are less at peace with death; perhaps they feel they would be letting their families down; or perhaps a relatively simple intervention could add another decade or more of life.

It is interesting that <20% of patients in this study received specialist palliative care, and those who did largely experienced this in the last month of life and died of cancer. Only 4% of the patients who died of a cardiovascular cause saw a palliative care specialist in the last month of life, which should raise concern, as it strains credulity that patients with such high resource utilization did not have unmet palliative care needs. In contrast to its prevalence in cancer care, palliative care is much less integrated into cardiovascular medicine.[14] It is unknown how palliative care may improve ACHD care, neither is it known at what point in the disease trajectory specialist palliative care is best utilized. Further, the concept that palliative care can minimize unnecessary or unwanted resource use only makes sense if there is a strong network of resources, staff, and infrastructure to support its success—a similar concern with other forms of multidisciplinary care. This also reflects the importance of ACHD clinicians' comfort with practising primary palliative care,[15] an easily developed skillset that can aid in eliciting and responding to patients' concerns. Primary palliative care practised by ACHD clinicians may be of particular importance for patients, such as those with complex CHD, who do not develop cancer or other conditions where they are likely to separately encounter specialty palliative care.

As we look toward future ACHD research needs, we must keep in mind the importance of first understanding patients' palliative care needs: what are their care preferences and expectations, illness understanding and their self-perceived ability to manage their care, and the impact of their symptoms on quality of life? We must work to determine what might be ACHD-specific goals of care, how these change as patients approach end-of-life, and how healthcare systems can best respond to and support patients' needs. Then, we must invest in the development and dissemination of interventions to target improvements in these areas of need. Examining the end of life for patients with ACHD provides a great opportunity to learn about the potential applications of palliative care in this unique patient population, guided by the experiences of those who have come before. This 'view from the end' provided by Van Bulck et al. can help chart future journeys.