Palliative care for people living with heart failure: European Association for Palliative Care Task Force expert position statement

Abstract

Contrary to common perception, modern palliative care (PC) is applicable to all people with an incurable disease, not only cancer. PC is appropriate at every stage of disease progression, when PC needs emerge. These needs can be of physical, emotional, social, or spiritual nature. This document encourages the use of validated assessment tools to recognize such needs and ascertain efficacy of management. PC interventions should be provided alongside cardiologic management. Treating breathlessness is more effective, when cardiologic management is supported by PC interventions. Treating other symptoms like pain or depression requires predominantly PC interventions. Advance Care Planning aims to ensure that the future treatment and care the person receives is concordant with their personal values and goals, even after losing decision-making capacity. It should include also disease specific aspects, such as modification of implantable device activity at the end of life. The Whole Person Care concept describes the inseparability of the physical, emotional, and spiritual dimensions of the human being. Addressing psychological and spiritual needs, together with medical treatment, maintains personal integrity and promotes emotional healing. Most PC concerns can be addressed by the usual care team, supported by a PC specialist if needed. During dying, the persons’ needs may change dynamically and intensive PC is often required. Following the death of a person, bereavement services benefit loved ones. The authors conclude that the inclusion of PC within the regular clinical framework for people with heart failure results in a substantial improvement in quality of life as well as comfort and dignity whilst dying.

1. Introduction

Heart failure (HF) affects about 1–2% of the general adult population in high-income countries.¹ At least 5% of this group suffers from symptoms at minimal exercise or at rest [Class III or IV of HF according to New York Heart Association (NYHA) classification] despite optimal cardiologic management.^2–4 People with advanced HF have a risk of premature death (80% of people with advanced HF die within 5 years) and report physical symptoms, psychosocial burdens, and spiritual needs similar to those reported by people with other advanced diseases, including cancer.^5–7

Scientific societies representing palliative care (PC), including the European Association for Palliative Care (EAPC), and cardiology, including the European Society of Cardiology recommend PC for people with advanced HF in order to improve their quality of life (QoL) and dying.^1,8–14 QoL has hardly been considered as an endpoint in large multicentre randomized trials, and the added value of PC provided along with optimal cardiological treatment has only been shown recently in one single centre randomized study.¹⁵ Access to PC throughout the whole course of HF, accordingly to needs, has been advocated.¹⁶ The World Health Organisation has recently recognized access to PC for all people who need it as an essential criterion of Universal Health Coverage.¹⁷ Although heart disease has been included in the 20 health conditions most commonly resulting in either death or suffering severe enough to require PC intervention,¹⁸ and circulatory disease is the leading cause of death (34% of all deaths),¹⁹ only a minority of people with HF across Europe receive PC^20–22 (merely 7% HF decedents compared with 50% of cancer patients had their PC needs recognized, and <1% of patients dying in hospices have HF as the primary diagnosis),²³ for a very short time (the mean time from PC referral to death is <2 weeks; significantly shorter than for people with cancer).²⁴ Improving access to PC for people with advanced HF might reduce their suffering and that of their loved ones, as well as decrease hospital readmissions.^15,25–29

The Board of the EAPC, recognizing the urgent need to improve the provision of PC for people with HF, has endorsed the initiative of professionals providing such care and approved the Task Force on Palliative Care for People with Heart Disease.³⁰ A group of multi-professional experts working in the field of PC and cardiology (physicians, nurses, ethicists, allied health professionals, and spiritual carers) from 10 countries has been charged on behalf of the EAPC with evaluating the existing data and current clinical practices with respect to PC for people living with HF. This position statement presents agreed opinions of these experts and has been approved by the EAPC Board for publication. The following topics will be discussed: basic definitions, symptom assessment, triggers for initiating PC, symptom management (breathlessness, pain, depression, and anxiety), advance care planning, spirituality and whole person care, addressing ethical dilemmas, adjustment of medical therapy, care for dying, and PC services.

2. Basic definitions

A variety of terms meaningful for this article are used internationally with varying interpretations. This, and a misperception amongst the lay public, patients, their families, and non-PC clinicians, often leads to belief that PC is only relevant to the last few weeks or days of life. This misperception is a major barrier to access PC for people with heart disease. A main aim of this article is to facilitate the correct understanding of the broad nature and wide applicability of PC for people living with HF. For clarity, key definitions for this document are therefore presented:

Palliative care is the active, total care for a person with incurable disease; that is, disease which may still respond to disease-modifying treatments, but is nonetheless progressive and life-shortening.³¹ PC is neither limited to a specific diagnosis, nor to a particular prognosis, and even if decline trajectories and patient characteristics differ among specific disease, the principle of PC, focusing on the improvement of the QoL, is universal. Ideally, PC should be introduced early on in the disease trajectory and increased as the disease progresses¹ or reduced/withdrawn if the condition improves (Figure 1). PC addresses symptoms and social, psychological, and spiritual problems.³¹ It affirms life and regards dying as a normal process; it aims to neither hasten nor postpone death. PC should be provided alongside optimal disease specific management and care.^1,8,32

Hospice care is a specific form of PC service dedicated to the whole person who approaches death and for those who love her/him. Care is focused on the individual’s needs and personal choices, striving to offer freedom from pain, dignity, peace, and calm. It is not primarily aimed at saving life or finding a cure.³³ In Europe, hospice care is often provided by freestanding centres offering care in the patient’s home or in a home-like in-patient setting. In the USA, a hospice is a specific insurance benefit dedicated to people who forgo life-prolonging therapies. In several countries ‘hospice care’ is used interchangeably with ‘specialist PC’. This article refers to a broad spectrum of PC.

End of life (EoL) is the period preceding a person’s natural death, characterized by the progression of a disease, which cannot be arrested by medical treatment. Depending on the person, her/his characteristics, the underlying disease and comorbidities different durations of expected survival are understood in the literature and policy in different countries as EoL—from several months until the last few days of life. EoL should be distinguished from active dying—a short period preceding imminent death, characterized by the waning of the physiological functions of a person and, limited to the last days or hours of life.³⁴

Transition of care is a change in the place, level, or goals of care. The main domains of care goals can be: cure, prolonging survival, optimizing function, improving comfort, achieving life goals, and supporting the family/caregiver. Transition of care is common and important during advanced disease.³⁴

Heart failure is the common end-pathway of many structural and functional cardiac diseases, which impair the ability of the ventricle(s) to fill and/or eject the blood. It is a clinical syndrome characterized by typical symptoms (e.g. breathlessness, ankle swelling, and fatigue) that may be accompanied by signs (e.g. elevated jugular venous pressure, pulmonary crackles, and peripheral oedema) caused by a structural and/or functional cardiac abnormality.¹

Comprehensive HF care is the integration of PC with guideline-directed HF management applied concurrently, with the focus varying according to needs, which change across time. It should be available to affected people and their caregivers throughout the whole course of patient’s HF journey.³⁵ (Figure 1).

Advance care planning (ACP) is a process that enables individuals to define goals and preferences for future medical treatment and care, to discuss these goals and preferences with family and healthcare providers, and to record and review these preferences if appropriate,³⁶ for the case of the loss of decision-making capacity.

3. Symptom assessment

The symptoms suffered by people with HF can be caused by HF itself [e.g. breathlessness, fatigue, and weakness (Figure 2)], comorbidities (e.g. musculoskeletal pain) and patients’ general condition or treatment side effects (e.g. deconditioning, nausea, constipation, depression, anxiety, sleep problems, confusion, and delirium).^25,37,38 Symptoms that persist despite optimal guideline-directed cardiologic treatment should trigger a PC approach or involvement.³⁹ These symptoms should be treated with as much attention as improving heart function and prolonging survival. This is particularly pertinent in advanced disease, including those awaiting transplantation or ventricular assist device (VAD) implantation.^40–42 Several symptoms that cause substantial suffering (e.g. depression, fatigue, tiredness, and poor appetite) may be regarded as unimportant given the seriousness of HF and, as a result, may be unaddressed. To ensure that patient-relevant causes of distress are identified and addressed, symptoms should be assessed systematically.⁴³ Extending the usual history by validated assessment tools increases the identification of symptoms 10-fold.⁴⁴ Any symptom can be assessed using the Numeric Rating Scale (NRS). Multi-symptom [the Edmonton Symptom Assessment Scale (ESAS)],^6,43,45,46 or multidimensional [the Integrated Palliative care Outcome Scale (IPOS)⁴⁷] tools can be used to assess symptoms comprehensively. Repeating the assessments helps quantify changes in symptom burden and the effectiveness of treatment. The simplicity and widespread distribution of the ESAS favours its broader use in clinical practice. The burden caused by a given symptom depends on its intensity, impact on functional capacity, and on QoL. For breathlessness, unpleasantness (reflecting affective distress it causes) should be assessed additionally to its intensity.⁴⁸ Anxiety and depression can be assessed using the Hospital Anxiety and Depression Scale (HADS).^1,49–51 Disease specific tools like the Kansas City Cardio-myopathy Questionnaire (KCCQ) (for which there is a short-form of 12 questions) or PC-specific like Functional Assessment of Chronic Illness Therapy–Palliative Care scale (FACIT–Pal), can be used to evaluate factors that limit wellbeing and monitor the efficacy of management.^15,45

4. Triggers for initiating PC provision

HF guidelines reinforce the focus on PC as an option for patients with advanced/end-stage HF, or those who are at the EoL, indicating however that ideally, PC should be introduced early in the disease trajectory and increased as the disease progresses,^1,8 so a needs and symptoms assessment-based approach is a more suitable model for integration of PC into cardiac care, than recognizing EoL.⁵² Reliance on prognostication as a trigger for the consideration of PC is ineffective due to the poor utility of current prognostic tools⁵³ and because PC needs do not correlate with prognosis.⁵⁴ Prognostic uncertainty should trigger rather than block assessing of PC needs.⁵⁵ As discussed above, symptom assessment tools may be helpful in this regard.⁵⁶

Conversations about the goals of care, assessment of PC needs and considerations of the need to include PC in the ongoing care can be initiated at annual HF review visits in less advanced stages of HF, or after each significant health-related events in more advanced stages⁵⁸ (Figure 3). Triggers for a PC approach or consultation should include distressing symptoms, existential distress, recurrent HF exacerbation, and progressive frailty or caregiver concerns.

PC integration should start when the symptoms and problems begin to emerge and persist and be provided alongside disease modifying treatments. A needs assessment tool validated for people with HF: the Needs Assessment Tool: Progressive Disease-HF (NAT: PD-HF) can help to identify people who can benefit from PC.⁶⁰ This tool includes four major sections: the patient’s wellbeing; the caregiver’s ability to provide care; the caregiver’s wellbeing; and issues that should raise consideration of referral to specialist services such as the lack of a caregiver.⁵⁶ Each section has a series of prompts, which act as an aide to assess the level of concern about unmet needs (none, some, significant) and identify the action taken by the clinician (managed by the clinician, managed within the team, referral to other resources). People with HF are deemed to have important needs if they were assessed as having ‘significant concern’ on any of the NAT:PD‐HF patient wellbeing domains.³² This assessment tool is designed to be completed in less than a minute at the end of a consultation and, in cancer patients, has been shown to reduce the level of unmet needs without prolonging consultations. It has recently been translated and validated in Dutch, and translations into other languages are in progress.⁶¹ Supportive and PC Indicators Tool (SPICT) can help to identify people who may have PC needs based on the risk of deterioration and dying, although it neither identifies what those needs are nor triages corresponding action.⁶² A combination of tools allows for a variety of triggers to flag the need for re-negotiating goals of care.

Basic PC related definitions and key principles of PC relevant for care for people with HF are summarized in Table 1.

5. Symptom management

5.1 Breathlessness

Breathlessness (dyspnoea) is the subjective experience of breathing discomfort or difficulty in breathing that consists of qualitatively distinct sensations that vary in intensity.⁴⁸ It can be acute, chronic, or episodic with the episodes usually superimposing on chronic (constantly present with usual fluctuations) difficulties in breathing. Episodic breathlessness is a severe worsening of breathlessness intensity or unpleasantness, beyond the usual fluctuations in the patient’s perception.^63–65 Breathlessness that persists despite optimal treatment of the underlying pathophysiology and results in disability is defined as a chronic breathlessness syndrome, which requires symptomatic management.^66,67 Breathlessness is reported by almost 90% of people experiencing advanced HF and is usually present at minimal exertion or at rest, substantially limiting the patients’ QoL and daily activities such as bathing or dressing.⁶⁸ Chronic breathlessness in people with HF can be related to haemodynamic status, skeletal myopathy and sarcopenia, chronic or acute comorbidities.^16,69 In the case of breathlessness exacerbation, potentially reversible aggravating factors should be sought and if appropriate, specifically treated. After or parallel to optimizing the guidelines recommended treatment of HF (re-establishing and/or maintaining optimal volume status)^1,9,12,14 and/or concomitant disease, non-pharmacological and pharmacological symptomatic treatment should be pursued. Most studies on symptomatic/palliative breathlessness management have been performed on unselected cohorts, so conclusions with respect to efficacy and safety specific in a HF population should be drawn with caution. Appropriately tailored exercise helps to improve functional capacity and skeletal myopathy. A physiotherapeutic approach, including breathing training, neuro-electrical leg muscle stimulation, or use of a hand-fan and walking-aids are considered as potentially helpful. Relaxation, breathing-relaxation training, and psychological interventions can be tried.⁷⁰ Oxygen therapy can ameliorate breathlessness in hypoxemic patients, but the data do not support use in those who are only mildly hypoxemic or normoxaemic.⁷¹ Pharmacological symptomatic treatment of breathlessness is based on opioids. The evidence for their efficacy is strongest for people with stable chronic obstructive pulmonary disease (COPD).⁷² The beneficial effect of opioids on breathlessness probably does not depend on its aetiology,⁷³ but data on the short-term use of opioids in people with HF are conflicting and data for longer-term use are promising, but inconclusive.^74–76

Most studies on symptomatic breathlessness management have been performed using oral low-dose morphine. Oral low-dose sustained release morphine has recently had its licensed indication extended to chronic breathlessness due to COPD, HF or cancer by the Therapeutic Goods Administration of Australia. Therefore this is the only drug and drug preparation anywhere in the world with a license for use in chronic breathlessness. Although the studies of morphine in HF give conflicting findings, based on the licensed dosing schedule morphine should be started at 10 mg per day, given to provide a steady state according to preparation (2.5 mg immediate release regularly four times daily; 5 mg modified release twice daily or 10 mg modified release once daily). In a dose increment study of people with a range of causes of breathlessness, a clinically important improvement occurred in 63% participants, 67% of responders benefited by 10 mg per day, 25% and 8% required dose escalation to 20 mg or 30 mg of morphine per day respectively.^77,78 After the start of treatment or dose increment, the initial response, if present, is seen in the first 24 h, however the magnitude of improvement may grow during a week (up to doubling the effect). If the response is inadequate, dose increases should not occur for at least one week. Doses can be titrated to a maximum of 30 mg/24 h of oral morphine (or equivalent dose of other opioid) if appropriate; a dose which appears to be unrelated to excess mortality or hospital admission, at least in people with severe COPD.⁷⁹ In significant renal impairment (Stages 4 and 5 of chronic kidney disease i.e. GFR <30 mL/min), something quite common among people with advanced HF and older adults, morphine should be avoided, used with caution, and/or switched to another opioid not having active metabolites with renal excretion.⁸⁰ Other opioids are sometimes used for breathlessness management, but there are no published adequately powered, placebo-controlled data in people with HF for this indication for any, other than oxycodone, where there was no benefit over placebo.⁷⁴ Phase 2 studies with fentanyl show promise.⁸¹ It is unknown if the alleviation of breathlessness is a specific feature of morphine or a class effect.

The most recent Cochrane review of benzodiazepines demonstrated a lack of evidence either for or against benzodiazepines. None of the published studies were done in people with HF. As the use of benzodiazepines has been associated with increased risk of all-cause mortality in severe COPD and other morbidity such as falls, caution is advised. Benzodiazepines, if at all, should be used as second- or third-line therapy, in acute episodes when other measures have failed and anxiety significantly aggravates distress.^79,82–85

5.2 Pain

Pain is present in most patients with advanced HF. The prevalence of pain increases with age and functional class, reaching 89% in those with NYHA Class IV HF.^86–88 At least moderate pain is reported by 61% of hospitalized HF patients, and pain at more than one site by 40%.^6,89 Chronic pain, if inadequately treated (which occurs more frequently for people with HF than for those with cancer⁹⁰), degrades QoL^87,91 and correlates with fatigue and depression.⁹² Untreated pain is also associated with more frequent hospital admissions due to HF-decompensation.⁹³ The involvement of PC services improves the burden of pain in in- and out-HF-patients.^26,89 Pain can be of cardiac (ischaemic) or non-cardiac (musculoskeletal, or caused by dyspepsia, gout, peripheral vascular disease, oedematous legs, or tense ascites) origin.^94–97 Appropriate management should be based on the likely pathophysiological mechanism of the pain, such as neuropathic, ischaemic, nociceptive, or inflammatory (for review see reference 98). Cardiac ischaemic pain is usually controlled with anti-anginal medication, but there are patients for whom this remains a severe problem despite optimal cardiologic treatment. Spinal cord stimulation might be considered in chronic refractory angina.^99–102 Intravenous strong opioids, such as morphine, are recommended for the relief of severe anginal pain related to acute coronary syndromes.¹⁰³ Opioids slow gastric emptying and might delay the absorption of orally administered antiplatelet agents. Administration of crushed tablets, prokinetic drugs, or parenteral loading are proposed to overcome this undesirable effect.^104–106

For chronic non-cancer pain, non-pharmacologic and non-opioid pharmacologic therapy are preferred.¹⁰⁷ Non-steroidal anti-inflammatory (NSAIDs) drugs increase fluid retention and should be avoided in patients with HF. Previously stable patients started on a NSAID have an increased risk of worsening HF.¹⁰⁸ Many people with HF also have renal dysfunction and take a loop diuretic and ACE inhibitor—adding NSAID increases renal strain, particularly in the older adult. Paracetamol appears to be safe in HF.¹⁰⁹ Topical NSAIDs might be tried; however, their safety has not been studied in HF patients. Data regarding the long-term use of strong opioids in chronic non-cancer pain are mixed overall and very limited in people with HF, and the risk of side effects and addiction should be carefully balanced in the decision to prescribe them. The opioids, if appropriate should be considered if pain persists despite non-pharmacological and non-opioid pharmacologic therapy and in the lowest dose for the shortest duration. In people with severely impaired renal function opioids with a safer metabolic profile, such as methadone, buprenorphine, or fentanyl, are preferred.

5.3 Depression and anxiety

The prevalence of depression among people with HF, especially in its advanced stage, is significantly higher than in the general population (up to 42% and 70% respectively vs. 20%).^49,110 Depression is an important, modifiable risk factor of HF-related hospitalization and death, and independent negative prognostic indicator. It also contributes to overall poor QoL.^{1,49,110–112} The co-existence of depression results in poor self-care, decreased medication adherence, increased smoking, and decreased activity leading to deconditioning and weight gain.⁴⁹ In contrast, anxiety often considered in connection with depression, does not seem more prevalent in HF than in the general population and does not pose the same risks as depression.^{49,110,111,113,114} Anxiety is associated with poor physical functioning due to the inability of individuals to implement effective coping strategies.¹¹⁵

Depressive symptoms may overlap with HF symptoms, making the diagnosis of depression more complicated.¹¹⁶ A vicious cycle ensues between depression and HF. Depression causes the activation of the hypothalamus-pituitary-adrenal (HPA) axis, resulting in elevation of cortisol level.⁴⁹ Due to the association of depression with reduced functional performance and HF instability, it is recommended that all HF patients be assessed for depression and treated if appropriate.^{92,114,117,118} To date, there are no published guidelines on the treatment of depression for people with HF but a number of approaches have demonstrated the improvement of depressive symptoms, physical function, QoL, and self-management skills, but not overall outcome. An integrated approach from a multidisciplinary team is recommended. Cognitive behavioural therapy and aerobic exercise training seem to give promising results. Pharmacological interventions may be necessary for some patients to treat depression but also to inhibit excessive activation of the neuroendocrine HPA axis related to the depression.⁴⁹ The selection of efficient and safe antidepressants, however, is challenging. Selective serotonin uptake inhibitors (SSRI) and alfa2-antagonists (mirtazapine) are thought to be the safest group of antidepressants for patients with HF, but the evidence is limited.¹ However, similarly to Monoamine Oxidase Inhibitors (MAOIs), they can cause hypertension.⁴⁹ Tricyclic antidepressants (TCA) can provoke orthostatic hypotension, worsening of HF and arrhythmias, and should be avoided in HF.^1,49 TCA and several SSRI (like citalopram) and mirtazapine can cause the prolongation of QT interval predisposing the development of ventricular tachycardia.⁴⁹

Elements of PC management most relevant in care for people with HF are summarized in Table 2.

6. Advance care planning

ACP is an essential component of PC, it increases the completion of advance directives, discussion of EoL preferences, improves the concordance between preferred and received care and might decrease rehospitalizations at the EoL.^119,120 Yet, ACP is often not done or poorly conducted in patients with HF. Preferences for life-sustaining treatments are often not discussed and documented resuscitation orders may differ from patient preferences.^121,122 Clinicians caring for patients with HF report as important barriers to ACP: reluctance of patients or family members to accept a poor prognosis, difficulty of patients or family members to understand limitations or adverse effects of life-sustaining treatments, and discordance among family members about the goals of care.¹²³ Patients and family members report as important barriers to ACP: uncertainty about care they would desire, a preference to concentrate on staying alive than talking about EoL care and uncertainty about which doctor is responsible for EoL care.¹²⁴ Overcoming these barriers is important for the delivery of high-quality PC. In fact, ACP leads to open communication and may give patients feelings of relief and more control about their care.¹²⁵ ACP can be initiated at any stage of a person’s life.³⁶ HF has an unpredictable trajectory. Moreover it increases the risk of cognitive impairment.^126,127 Therefore, timely ACP, when the patient is able to participate in decision-making process should be a standard element of clinical care of people with HF and ACP should not be postponed until a patient approaches the end stage of his or her disease.³⁹ Nevertheless, identifying the appropriate moment in the course of the disease trajectory to start ACP can facilitate the process.¹²⁸

ACP should be considered at transition points during the course of the disease like hospital admission, symptom burden or functional decline despite optimal disease specific treatment, and the exhaustion of disease-oriented treatment options.¹²⁸ ACP should to be adapted to the patient’s readiness to engage in ACP and should not be limited to discussing and recording life-sustaining treatment preferences.³⁶ Indeed, communication about the goals of care and addressing the concerns of the patient and loved ones about the EoL are paramount. Communication with PC consultants or teams can help to identify or refocus goals of care.^53,129,130 Furthermore, previously documented goals of care and preferences regarding (life-sustaining) treatments and care should be updated regularly.^131,132 Disease-specific aspects need to be addressed where appropriate, such as fear of dyspnoea at the EoL, reprogramming of an implantable cardioverter-defibrillator (ICD), so that it does not deliver shocks or withdrawing mechanical circulatory support delivered by VADs in the dying phase.¹³³

7. Whole person care and spirituality

The ‘Whole Person Care’ concept is based on treating the patient as an integral human being consisting of an inseparable body, mind, and spirit. Providing the very best medical service adjusted by psychological and spiritual care ensures that the person is addressed as an integral individual, even if optimally fixed medical issues cannot cure the disease. Addressing medical, psychological, and spiritual needs facilitates the process of growing personal integrity in response to even incurable disease or injury. This growth of the sense of integrity or wholeness is known as the process of healing. This concept recognizes the meaning of relations between all those participating/involved in the care for a person: a patient her- or himself, her or his family, friends and caring team,^134,135 and reinforces the role of spirituality in a person’s life in addition to physical and mental dimensions.

Spirituality has a multidimensional nature, and encompasses existential questions, values, and religious matters.¹³⁶ Although spirituality is recognized as one of the four dimensions in PC, research in this area is underdeveloped in terms of people with HF. Studies have shown that spirituality is an important and integral component of QoL and affects the person’s ability to place their difficulties in perspective.¹³⁷ The spiritual needs of people with HF and their carers are influenced by hopelessness, isolation and altered self-image (loss of confidence, dependency, being a burden) associated with chronic illness and disability.¹³⁸ In advanced HF, spiritual wellbeing remains stable over time and varies according to race and symptom distress.¹³⁹ Social and psychological decline both tend to track the physical decline, while spiritual distress fluctuates independently.¹⁴⁰ Experiencing spiritual peace better predicts mortality than functional status and comorbidity¹⁴¹ and greater spiritual well-being is associated with a lower incidence of depression.¹⁴² Hope and hopelessness are constructs conceptually linked with depression and spirituality. One study indicates that expression of hope positively affects cardiovascular outcomes.¹⁴³ Adjunct spiritual counselling appears to have a positive impact on QoL.¹⁴⁴

Besides the openness to the spiritual dimension of the patient, the whole person care approach focuses the clinician additionally on curing the illness, acknowledging the simultaneous process of internal healing, i.e., ‘becoming psychologically and spiritually more integrated and whole; a phenomenon which enables persons to become more completely themselves and more fully alive’.¹⁴⁵ As the relationship between clinician and the patient has a mandatory meaning for healing, medical professionals should be therapeutically present (be on hand i.e. ‘here and now’); enhance the patient's dignity and his or her sense of being a unique human being; be open to the spiritual needs of the patient and cooperate with specialists in spiritual care such as chaplains; be ready to assist in the patient's quest for meaning; and take care of her- or himself (including self-development, as a person and as a professional).

Spiritual care, which addresses an essential aspect of humanity, should be integrated into care for people with HF within a wide range of interventions and attitudes (from the therapeutic presence of clinicians to the professional help offered by specialists in spiritual care/chaplains, pastoral care workers).¹⁴⁶

8. Addressing ethical dilemmas

Four ethical principles encompass dilemmas that arise during the care of people with advanced HF: beneficence, non-maleficence, respect for patient autonomy, and justice.¹⁴⁷

Beneficence refers to the clinician’s duty to act for the good or benefit of patients. Non-maleficence refers to the clinician’s duty to prevent or avoid harming patients. Clinicians should weigh the anticipated benefits and harms of tests and treatments in the context of the patient’s prognosis and health care-related goals. ‘Double effect’ is a well-established concept that allows clinicians to prescribe potentially harmful medications, procedures, or other treatments if the intent is good, the harmful effect is not intended, and the potential benefit of the treatment outweigh the harms.^148,149

Respect for patient autonomy requires that clinicians inform patients about their diseases and prognoses and the risks, benefits and alternatives to tests and treatments. Regarding patients with advanced HF, clinicians should inform those with implantable cardiac devices (e.g. ICDs) of the option of withdrawing device therapies or ‘device’s function deactivation’ (e.g. reprogramming an ICD so that it does not deliver shocks).^150,151 Clinicians should ensure patients’ decisions to refuse or request the withdrawal of therapies are informed and respect such decisions.¹⁵¹ Respect for patient autonomy also underlies the process of ACP; clinicians should encourage patients with advanced HF to articulate and document their health care-related values, goals, and preferences.¹⁴⁹

Justice requires that clinicians base their testing and treatment recommendations on medical evidence and need, not on patient-specific characteristics (e.g. race and sex).¹⁵²

Ethical dilemmas that arise when caring for patients, occur when two or more of the aforementioned ethical principles conflict with each other. In patients with advanced HF decision making regarding withholding or withdrawing life-sustaining treatments commonly precipitates these dilemmas. For example, if a patient’s request for deactivation of an implantable cardiac device (i.e. withdrawal of device therapies) conflicts with the clinician’s perceived beneficence and non-maleficence duties, the clinician should strive to resolve the dilemma (e.g. care conference). For situations in which such dilemmas cannot be resolved, ethics consultation and/or PC consultation should be considered. There are multiple approaches to ethics consultation.¹⁴⁹ However, most involve systematically reviewing the patient’s medical situation and health care-related values, goals and preferences, QoL concerns, contextual features associated with specific case, and other factors. This approach usually defines the ethical dilemma and suggests a solution to resolve it.

9. Adjustment of medical therapy

Transition of the goals of care towards improving comfort and focusing on alleviating symptoms requires compassionate communication with patients and their families and loved ones and should be connected with a review of ongoing therapies with respect to their applicability. The validity of former indications for their use, after setting new goals, should be evaluated. Continuous optimization of HF therapy should be pursued, if only possible.^1,9,14 Treatments relevant for symptom management or prevention should be continued if well tolerated and the dose regularly reviewed. Medicines prescribed for indications that are becoming no longer relevant should be considered for withdrawal. Therapies causing undesirable side effects and preventive drugs, especially those with a long delay in showing their benefits, such as statins, should be stopped.¹⁵³ However, routinely stopping any HF treatment when starting PC is inappropriate as many HF treatments, like angiotensin-converting enzyme inhibitors (ACEI) and angiotensin II type I receptor blockers (ARB) or angiotensin receptor-neprilysin inhibitors (ARNIs) as they may be important for symptom control.¹ Diuretics help maintain euvolaemia and control breathlessness and should not be withdrawn unless there is a clear reason to do so.¹⁵⁴ If the patient’s condition deteriorates and fluid intake decreases, diuretic dose reduction could be appropriate. ACEI, ARB, or ARNI may help prevent pulmonary congestion but can cause symptomatic hypotension or the worsening of renal function. Dose reduction or discontinuation should be individually tailored.¹⁵⁵ Beta-blockers prevent tachycardia and/or angina, especially in patients with atrial fibrillation (and) prone to rapid ventricular rate. If they need to be reduced or stopped due to symptomatic hypotension or low cardiac output, this should be done gradually and digoxin may become an alternative.¹⁵⁵ In the case of significant bradycardia, beta-blockers should be reduced or stopped. Inotropic drugs may provide symptomatic benefits in advanced HF as part of a PC approach.^1,14,156 The intermittent infusion of intravenous inotropes might sometimes be considered as PC intervention in inpatient institutions or even in home care to improve both symptoms and QoL.^157–161 Inotropic drugs should not be started or continued in patients who are actively dying as they usually no longer provide any symptomatic benefits in such situations.¹⁶² If HF status improves, re-adjustment of therapy is then needed, including both restarting or increasing previously reduced doses of HF medication, and/or reducing or withdrawing PC medications (e.g. opioids after breathlessness or pain has improved).

In patients with an active ICD, the option of reprogramming the device at the EoL, to avoid potentially painful and usually futile shocks should be discussed in advance and, if agreed, performed timely.^163,164 Anti-tachycardia pacing (ATP), which is generally well tolerated can be left active if the patient does not wish to deactivate all anti-tachyarrhythmic therapies or if it might be in the patient’s best interests. More complex are requests for pacemaker deactivation. Some patients fear that an active pacemaker can prolong dying. However, this therapy neither prolongs dying nor causes symptoms, furthermore anti-bradycardia pacing can improve QoL, even in patients who are dying, by preventing symptoms caused by low heart rate and/or pauses in heart rhythm (e.g. dizziness, presyncope, and breathlessness).^165–167 Likewise, discontinuing the resynchronization component should be avoided, as the loss of pacemaker-mediated synchronization can precipitate HF-related symptoms.^151,168 A multidisciplinary approach should be considered including PC and cardiology, and ethics if needed, when patients or their caregivers request deactivation of antibradycardia or resynchronization pacing.

10. Care for dying

About 60–70% of deaths in patients with HF have a cardiovascular cause, with HF-related death being either sudden or caused by progressive pump failure.^169–171 For this reason, a team caring for people with HF should be familiar with diagnosing dying, caring for the dying, looking after the bereaved, and communicating about these issues. Diagnosing dying should be based on a multidisciplinary team discussion, in order to align the clinical perceptions of different professions.^172,173 During the dying phase, progressive weakness and immobilization, loss of interest in eating and drinking, cognitive impairment with diminishing verbal communication, changes in breathing pattern, and existential suffering might occur.^174–176 Dying is a dynamic process characterized by changes in diagnostic appraisal, as well as physical, psychosocial, and/or spiritual needs of the patient and their family and loved ones.

Many people prefer to die at home, but 60–80% of all patients will die in institutions like hospitals, nursing homes, residential homes, and hospices.^177–179 The preference with respect to place of care whilst dying can change during the course of the disease.^180,181 The presence of a family caregiver who supports a patient’s wish to die at home is one of the most important factors enabling a home death.^{177,182–184} Therefore, counselling, support, reassurance, and encouragement of relatives should be a major component of psychosocial care for patients and their families.¹⁸⁵ Earlier ACP contributes to realizing patient preferences.^186–190

When dying occurs, moist breathing (a ‘death rattle’) and confusion are common.¹⁹¹ If for any reason ICD has not been deactivated previously, and unwanted ICD shocks occur, a magnet can be taped over the device generator to prevent further shocks and perform electronic deactivation if possible.^{150,151,192–195}

All diagnostic, therapeutic, and nursing interventions that do not contribute to the aim of preserving the highest level of comfort should be discontinued or not initiated.^185,196

Palliative Care interventions most relevant in supporting people living with HF are summarized in Table 3.

11. PC services

An interdisciplinary approach encompasses the patient, her or his family, and loved ones and addresses PC needs wherever the patient is—at home or in institution. PC is divided into generic and specialist PC.^18,31 Generic PC, termed the ‘PC approach’, is provided by all health professionals, who have basic PC training and incorporate PC principles into routine patient care. Specialist PC is provided by a multi-professional team for whom PC is the core practice and who has specialist training in PC. Specialist PC is needed for patients with needs or problems that are complex and/or persist despite generic PC.¹⁹⁷ PC should be provided alongside optimal disease specific management and care.^1,8,32

The complex nature of the needs and symptoms experienced by people with HF require multi-disciplinary collaboration between cardiology and PC.^1,198 Both HF and palliative management need to be regularly reviewed and optimized. Most PC concerns should be within the skills of the usual care teams (cardiology, primary care, care of older adults), supported by a specialist PC for education, training, and clinical care if needed. For this reason, it could be reasonable to have a PC team, or at least a PC specialist in the cardiac team, and a cardiologist in the PC team. The PC team usually encompasses physicians, nurses, allied health professionals (AHPs), and chaplains, sometimes being supported by pharmacists and ethicists. The term AHP includes psychologists, pharmacists, physiotherapists, dietitians, speech and language therapists, occupational therapists, and social workers. Each discipline/profession plays a role in addressing patients’ and relatives’ PC needs. AHPs provide a rehabilitative approach, energy conservation techniques, enablement, self-management, and self-care.¹⁹⁹ Physiotherapy and occupational therapy-led interventions are especially important for non-pharmacological breathlessness management^199–201 and the improvement of functional ability.^202–204 Occupational therapists have core skills in non-pharmacological fatigue and anxiety management, along with the assessment and provision of equipment to maintain function and optimize QoL.²⁰⁵ PC provides as well bereavement service to support these who lost a loved one.

Despite the promising evidence supporting the involvement of multi-disciplinary PC in the care of people with HF,^15,26,28,29 there have been no trials to test which model of service provision is the most effective, although the use of an integrated approach based on the identification and triage of problems is promising⁵⁵ Services may be led by cardiologists, PC specialists, or jointly, and include common core components of care.^206–211

12. Conclusions

PC improves the QoL of many people living with HF, their families and loved ones. For most, generic PC along with optimal HF care is all that is required and can be provided by the core team (cardiology, primary care, or care of older adults) with access to specialist PC as needed. The most important step in the improvement of PC provision for people with HF is the proper understanding of a broad spectrum of PC services and the recognition of the PC needs they have. Symptom management, support in decision-making, communication including disease progression and EoL issues, advance care planning as well as addressing psychosocial and spiritual problems are the fields PC is focused on. PC should complement cardiologic care, and not be seen as an alternative to it. A responsive integrated cardiac and PC for all patients with HF when needed regardless of prognosis should be the landmark of modern comprehensive care for people with heart disease.

Disclaimer: The editors acknowledge that this position statement represents the opinion of the author and does not represent the opinions of the European Society of Cardiology, nor is it endorsed by the European Society of Cardiology.

Acknowledgements

The authors thank Lourdes Rexach from the Unidad de Cuidados Paliativos Hospital Universitario Ramón y Cajal, Madrid, Spain, and Luis Parente Martins from the Centro Hospitalar Lisboa Norte-Portugal for their participation in the reviewing process of the position statement. The authors also thank Louise Bellersen from Radboud University Nijmegen the Netherlands, David Oliver from the University of Kent, UK, Otmar Pfister from the University Hospital in Basel, Switzerland, and Ruthmarijke Smeding from Switzerland for their contribution to the earlier stages of the Taskforce work.

Conflict of interest: The authors P.Z.S., B.A.-E., S.J.G., K.H., M.J.J., C.L., M.M.-S., M.M., S.T.S., E.S., and P.J.L. have nothing to disclose. D.C.C. reports he is an unpaid member of an advisory board for Helsinn Pharmaceuticals, is a consultant to Specialised Therapeutics and Mayne Pharma, and received intellectual property payments from Mayne Pharma. T.G. reports personal fees from Servier, Krka, Polpharma, Abbott, outside the submitted work. D.J.A.J. reports personal fees from Boehringer Ingelheim, Novartis, AstraZeneca, outside the submitted work. M.K. reports personal fees from Mundipharma, Takeda, Teva, Stada, Angellini, Molteni, Pfeizer, outside the submitted work. P.S.M. reports other fees from Boston Scientific Patient Safety Advisory Board, other from NEJM Journal Watch General Medicine, other fees from Medtronic Medical Education, outside the submitted work. M.R. reports she is member of International Committee of Medical Journal Editors.

References