Special circumstances and symptom management

Providers seemed least comfortable addressing internal cardioverter-defibrillator deactivation and familial disagreement. These are examples of complex ethical and social situations which may complicate medical and advance care planning decision-making and challenge the physician–patient relationship. Choosing to minimise aggressive care or limit life-sustaining treatment, including internal cardioverter-defibrillator deactivation, is a patient’s prerogative, even when providers or family members disagree.^{Reference Lampert, Hayes and Annas13–Reference Kramer, Matlock and Buxton15} Physical symptoms affecting quality of life are often what prompt patients to seek care, with the hope that the provider can offer alleviating treatments. Symptom management is a major component of palliative care, yet providers’ comfort in managing symptoms may vary. It is not surprising that our study suggests that providers may be most comfortable addressing physical symptoms, since this is the focus of cardiology training. However, providers rated their palliative care knowledge as 5.3 on a 0–10 scale, and this did not increase with years in practice, suggesting providers are not learning these skills with time. Thus, these findings present an excellent opportunity for provider education about the services palliative care can offer, and how and when to incorporate palliative care for more comprehensive and holistic patient care.

The current Core Cardiology Training Statements list knowledge of the management of psychosocial contributions to cardiovascular disease and knowledge of the principles and indications for palliative care as core competencies.^{Reference Halperin, Williams and Fuster16,Reference Warnes, Bhatt, Daniels, Gillam and Stout17} However, they do not direct hands-on exposure, and in a recent survey, cardiology trainees reported inadequate palliative care skills training.^{Reference Crousillat, Keeley, Buss, Zheng, Polk and Schaefer18} These training documents are more inclusive than prior iterations, as recognition of the importance of this type of care continues to grow, but until clear and comprehensive training recommendations for these issues exist, great potential for providers to avoid these important issues out of lack of knowledge or discomfort will remain.

Starting earlier and the need for a systematic approach

The early introduction of advance care planning and palliative care is now considered standard of care for cancer, and benefits to their use have been recognised in heart failure due to acquired disease.^{Reference Diop, Rudolph, Zimmerman, Richter and Skarf19} However, when patients have lived with a disease their entire lives, as they do with CHD, it can be difficult to recognise the optimal time and way to broach these discussions in order for them to be meaningful. This challenge is not unique to ACHD. Research in cystic fibrosis, another chronic illness with paediatric onset, has revealed that patients are interested in earlier advance care planning and palliative care integration, preferably while healthy.^{Reference Linnemann, Friedman and Altstein20,Reference Dellon, Helms and Hailey21} Cystic fibrosis providers echo the concerns that end-of-life discussions occur too late and that palliative care services are underutilised.^{Reference Braithwaite, Philip and Tranberg22} They also seek education regarding communicating with patients and families about these topics and managing the emotional burden of this responsibility.^{Reference Braithwaite, Philip and Tranberg22}

Uncertainty of prognosis is a major issue driving delays in advance care planning and palliative care discussions in ACHD.^{Reference Lin, Cohen and Bhatt23} The natural history of ACHD in the current care era remains poorly defined, and it is thus difficult to predict when a life-threatening event may occur. Extrapolation from other conditions or others’ experiences may be helpful but not sufficient. Provider comfort with discussing prognoses does not guarantee comfort with end-of-life discussions. In this study, it seemed that it was easier for providers to envision discussing advance care planning when patients had worsening symptoms or a hospitalisation, that is, an “event” outside of the status quo. The chance that a patient’s condition might improve with treatment was perceived as a barrier. Given this prognostic uncertainty, in addition to patients’ young age and everyone’s hope for a favourable long-term outlook, the risk is that providers may forego uncomfortable advance care planning and end-of-life discussions until patients are very sick.

However, the 2018 American College of Cardiology/American Heart Association Guidelines for the Management of Adults with CHD suggest that advance care planning and palliative care discussions should not wait until death is imminent.^{Reference Stout, Daniels and Chair24} This way, discussions can prompt patients to think about what is important to them before the arrival of critical moments, and patients and providers can work together to plan for care that aligns with patients’ goals. Educational efforts should target providers (such as the 22% of respondents in this study) who would delay conversations until the onset of severe illness. Age 18 or the transition to adult care has been recommended by patients and providers as appropriate times to begin advance care planning discussions, although the decision must be made on an individual patient basis.^{Reference Sable, Foster and Uzark5,Reference Deng, Gleason and Khan8} Further, because patients’ wishes may change, decisions should be revisited at regular intervals and certainly with any upcoming procedure or major change in health status, such as initiation or heart failure medications, hospitalisations, or change in exertional capacity.^{Reference Steiner and Kovacs25}

Comprehensive care requires a team

Complex medical and psychosocial situations may limit the likelihood that ACHD providers conduct advance care planning and palliative care conversations. Interestingly, in an open-ended question asking about case-specific facilitators and barriers to advance care planning, respondents described almost seven times as many barriers as facilitators, highlighting the often-daunting nature of this task. Furthermore, 20% of respondents were unsure whether there was a procedure by which advance care planning documents could be stored within the medical record, suggesting gaps in preparedness for initiating discussion of advance directives.

As the importance of quality of life and care preferences gain recognition in ACHD, palliative care efforts will continue to grow. In addition to education to enhance ACHD providers’ comfort providing primary palliative care, the best way forward may be a shared approach, with the creation of interdisciplinary care teams to help provide secondary or specialty palliative care. Working alongside traditional cardiology providers, teams may include nurses, social workers, and providers from related medical specialties to ensure all patient care needs are addressed. For example, dedicated nurse coordinators may assist with holistic patient assessment, including patient education and self-care management.^{Reference Sillman, Morin and Thomet26} Bioethicists may help define benefit, harm, and futility in the context of medical interventions, and they can assist providers, patients, and families in understanding each other’s perspectives.^{Reference Robinson, Cage and Erler27,Reference Wasson, Anderson, Hagstrom, McCarthy, Parsi and Kuczewski28} Palliative care specialist integration can lead to better symptom control and care continuity and coordination.^{Reference Siouta, Van Beek and Van Der Eerden29–Reference Smallwood, Thompson and Warrender-Sparkes31} In our current study, although 70% of respondents reported having access to palliative care consultative services, we did not ask whether services are easily accessible, which could account for the relative hesitancy to make referrals. Efforts to increase the availability of these resources, such as to decrease wait times or streamline consultation as part of another medical visit, can lead to earlier involvement. Using the combined expertise of interdisciplinary team members, care provided may be better aligned with patient and family goals.

Limitations

A few limitations to these findings must be considered. The estimated (conservative) response rate for this survey was lower than 20%. Although ours was similar to the response rate in other surveys of providers,^{Reference Clowse, Eudy and Kiernan32–Reference Shillinglaw, Viera, Edwards, Simpson and Sheridan34} it allows opportunity for selection bias. We cannot predict how non-respondents would have answered survey questions, but it is reasonable to hypothesise that respondents opted for study participation because they recognise the important of advance care planning, thus potentially biasing this sample more favourably to advance care planning discussions. The intent of this study was to assess providers’ views regarding palliative care based on their understanding of this type of care. Responses may have been different if formal palliative care training was provided or if vignettes included more information about palliative care interventions and case outcomes. In addition, this was largely a sample of American providers, the majority of whom have been in practice 10 years or less. Thus, generalisability to providers outside of North America remains unknown. Further, in this assessment of advance care planning discussions, we did not account for prioritisation of this amongst other issues that may take precedence during a time-limited patient encounter.^{Reference Arndt, Beasley and Watkinson35,Reference Dugdale, Epstein and Pantilat36} For example, a provider may feel compelled to spend visit time discussing physical needs and items that are required components of electronic health record documentation over advance care planning. Despite these limitations, results from this study are important to guide future research, including interventions.