Management of cardiac implantable electronic devices in patients receiving palliative care

INTRODUCTION — Discontinuation of cardiac implantable electronic device (CIED) therapy, including both permanent pacemakers (PPMs) and implantable cardioverter-defibrillators (ICDs), is a complicated issue. For many patients, these are often life-sustaining therapies, with many ethical, legal, and religious principles that underlie the decision-making process to withdraw CIEDs. The issues related to changes in CIED therapy, including specific issues related to palliative care, will be discussed here. A more extensive discussion of palliative care planning and ethical issues which arise in palliative care are presented separately.

●(See "Palliative care: The last hours and days of life".)

●(See "Overview of comprehensive patient assessment in palliative care".)

●(See "Ethical issues in palliative care".)

KEY CONCEPTS IN PALLIATIVE CARE — A few key concepts specifically relevant to the management of CIEDs in patients receiving palliative care will be reviewed here (algorithm 1).

Advance care planning — Patients at any stage of health or illness should be encouraged to engage in advance care planning to identify their goals of care, articulate wishes in the setting of serious illness, and name a surrogate decision maker. Advance care plans should also specifically address CIED management when the patient has one. Advance care plans and proxy designations can always be subsequently modified in the event of a change in clinical status or change in the patient's goals of care. (See "Advance care planning and advance directives" and "Discussing goals of care".)

Patients and advance directives — Advance care planning should be an important part of every patient's care plan regardless of underlying illness, but such advance directives take on additional importance for patients with significant medical comorbidities who are being considered for palliative care. Advance-care planning for patients with CIEDs however, still needs improvement, as evidenced by the following observational data [1-4].

●In a retrospective study of the prevalence of an advanced directive among 420 patients with an implantable cardioverter-defibrillator (ICD), only 28 percent had an advance directive in place at the time of ICD implantation, with only 1 percent specifically addressing the issue of ICD deactivation at end of life [1].

●In a survey of 278 patients with an ICD, more than half of the patients had executed an advance directive, but only 1 percent had included a plan for the management of the ICD [2].

●In a nationwide survey of 3067 participants with CIEDs from the Swedish ICD and Pacemaker Registry which asked patients 11 questions (true or false type questions) about their knowledge of the ICD and its function in relation to end-of-life issues, 29 percent of patients were able to answer only five or fewer questions correctly, indicating a significant lack of understanding of the ICD and how it might impact quality of life in an end-of-life situation [3].

Clinicians and advance care planning — Advance care plans should be an integral part of the clinical care plan for all patients, especially when caring for patients who are being considered for palliative care. However, patient and clinician understandings regarding the role of advanced directives and the capabilities of CIED programming can be vastly different [5-10]. As examples:

●In a survey of United States clinicians, one-third of internists and two-thirds of electrophysiologists who responded believed patients knew they could deactivate the shocking function of an ICD [5]. However, in a separate survey, nearly 50 percent of patients indicated that they had never considered ICD deactivation in the context of end-of-life situations [6]. Clinicians who believe that patients know the options for device management at the end of life may be less likely to discuss deactivation [5].

●In a survey of 294 patients in the Netherlands, 68 percent believed that it is possible to turn the ICD off [7]. In contrast, in a 54 patient study in the United States, only 3 percent recalled receiving information about deactivation when providing consent for implantation, and 38 percent became aware later [8].

●In one chart review of 150 patients who underwent device deactivation at a tertiary care center, 42 percent of patients with device deactivation had a palliative care consultation, and of those, 68 percent specifically addressed device deactivation, indicating the potential value of a palliative care consult regarding goals of care [9]. A separate study revealed that only 10 percent of hospice providers have a device deactivation policy [10].

When differing opinions or beliefs are present — Although patients have the right to request withdrawal of therapy, it is possible that the personal and professional values of the provider and the patient may differ. Various professional society guidelines stipulate that clinicians in this position have an obligation to arrange for alternative provisions of care in cases of conscientious objection that cannot be resolved by ethics or clerical consultation [11-13]. Additionally, while ethicists are not routinely involved in decisions regarding withdrawal of CIED therapy, there are select occasions in which an ethics committee may be helpful, namely when the patient is unable to provide consent and there is a difference of opinion among family members and/or health care providers. Separate topic reviews on discussing goals of care and handling requests for potentially futile or inappropriate therapies are available. (See "Discussing goals of care" and "Palliative care: Medically futile and potentially inappropriate therapies of questionable benefit".)

Withholding versus withdrawing therapy — Withholding therapy refers to not providing a therapy that may be indicated, in contrast to withdrawing therapy which generally refers to removal of a previously instituted and indicated therapy. Withholding a life-sustaining therapy is sometimes seen as emotionally easier for clinicians and families to accept compared with withdrawing a previously instituted life-sustaining therapy, probably because there is a perception of less involvement in the patient's death. However, in multiple countries, including the United States and United Kingdom, there is no ethically meaningful distinction between withholding and withdrawing life-sustaining treatments.

A more extensive discussion of the ethical issues in palliative care as well as the distinction between withholding and withdrawing therapy is presented separately. (See "Withholding and withdrawing ventilatory support in adults in the intensive care unit", section on 'Ethical misperceptions about foregoing ventilatory support' and "Ethical issues in palliative care".)

FREQUENCY OF LATE-LIFE ICD THERAPIES — Patients and providers should engage in early and frequent discussions regarding how the CIED fits in with the patient’s goals of care and advance care planning. Patient opinions regarding their end-of-life implantable cardioverter-defibrillator (ICD) therapy can vary widely. Some patients choose to deactivate their ICD when nearing end of life. Others wish to have all possible efforts at prolonging life; they may choose to maintain ICD therapies and accept the potentially negative side effects (eg, shock-related pain, anxiety, etc). For these patients, receiving ICD shocks near the end of life can have a profoundly negative impact on quality of life and may also negatively impact family and friends.

Unfortunately, patient and provider discussions regarding ICD deactivation occur much less frequently than do discussions regarding living wills and health care proxy assignment. In a prospective study of 51 patients with ICDs and significant medical comorbidities who were followed for up to 18 months, living wills and health care proxy assignment were completed 88 and 98 percent of the time, whereas communication about patient prognosis and ICD deactivation occurred only 10 and 23 percent of the time [14].

These low rates of discussions regarding ICD deactivation likely result in low rates of ICD deactivation near the end of life. A retrospective chart review of 98 patients with ICDs from the MADIT II who later died found that 15 percent chose to have their ICDs deactivated [15]. This is consistent with studies from other countries. In a study from Sweden, 52 percent of the patients had a do-not-resuscitate order, yet 65 percent of them still had the ICD activated 24 hours before death [16].

Unfortunately, ICD shocks are common at the end of life.

●Last month of life – Interviews with family members of deceased patients in a single practice found that approximately 20 percent of patients with ICDs had the device discharge in the last month of life [5]. A separate clinical cohort study from Japan studied 27 patients with do-not-resuscitate orders. Twenty-seven percent of patients experienced an ICD shock, and 24 percent experienced electrical storm [17].

●Last 24 hours of life – In the MADIT II trial, 83 patients had terminal illnesses and active ICDs; 12 percent of these patients had a device discharge within 24 hours of death [15]. In an autopsy study from Sweden, 125 ICDs were explanted from patients who had died. The study showed that 31 percent of patients received shock treatment during the last 24 hours of their lives. However, arrhythmic death was the primary cause of death in only 13 percent [16]. Most notably, 52 percent of the patients had a do-not-resuscitate order, yet 65 percent of them still had the ICD activated 24 hours before death [16].

INDICATIONS FOR DISCONTINUING CIED THERAPY — The discussion around discontinuation of CIED therapy is different depending on whether the device in question is a permanent pacemaker (PPM) or an ICD (algorithm 1). In general, however, a discussion regarding the discontinuation of CIED therapy typically arises in one or more of the following settings:

●The goals of care have changed – As examples:

•The patient and/or the healthcare proxy have decided (patient preference) that a patient with an advanced serious life-threatening illness should not be resuscitated by either external or internal (ie, ICD) defibrillation.

•The patient and/or the healthcare proxy have decided (patient preference) to withdraw treatments in a patient who is pacemaker-dependent.

●A CIED complication has occurred – Most commonly due to infection or device component malfunction necessitating CIED removal.

Patient-directed change in goals of care — The concept of patient autonomy underlies both the ethical and legal principles surrounding CIED deactivation, with these ethical and legal principles having been well established. Patients with capacity (or the patient's legally designated surrogate) can request discontinuation of any medical or device treatment, including therapies such as pacemaker treatment in a pacemaker-dependent patient. When made by a patient with capacity or the relevant surrogate, such a decision falls within the spectrum of "withdrawal of treatment", is ethically sound, and should be distinguished from an act of euthanasia or physician-assisted suicide. No patient is committed to therapy that he or she no longer wishes to receive [18]. In addition, it is not necessary for patients to be terminally ill to make these requests. (See "Ethical issues in palliative care".)

Device complications necessitating CIED removal — Occasionally, it becomes necessary to remove a CIED system, the generator, and accompanying lead(s) due to a complication, most commonly CIED system infection or structural failure of a CIED component such as lead fracture. When a pacemaker is removed, the indication for pacing versus the risks of reimplantation should be addressed. When an ICD is removed, the patient is no longer protected from sudden cardiac death, and a decision must be made about if and when to place a new ICD. (See "Cardiac implantable electronic devices: Long-term complications" and "Infections involving cardiac implantable electronic devices: Epidemiology, microbiology, clinical manifestations, and diagnosis".)

WHAT TO EXPECT AFTER DEVICE DISCONTINUATION

Discontinuation of ICD therapy — All modern ICD systems have the ability to treat ventricular tachyarrhythmias and provide for backup pacing functionality (ie, serve as a pacemaker). Each of these functions can be turned off individually. For most patients with an ICD, discontinuing antitachycardia therapies (including antitachycardia pacing and shocks) by reprogramming the device will not have any immediate impact on comfort or quality of life (algorithm 1). The only exception would be in a patient who is receiving, or has recently received, one or more ICD shocks. In this case, quality of life may improve by reducing painful shocks, but a recurrent ventricular arrhythmia would be untreated and potentially fatal.

When discontinuation of ICD therapy is requested because of a change in the goals of care, the role of the clinician is to fully explain the implications of the decision. Some issues that may be important include:

●Shock-related pain and anxiety – Among patients with a terminal illness and a poor prognosis, the pain from an ICD shock, or the anxiety of potentially receiving an ICD shock, can be contrary to a primary goal of maintaining the patient's comfort [19]. When a patient with a preexisting ICD has a new diagnosis of a terminal illness, the option of disabling ICD therapies should be included in broader discussions of end-of-life care. Many patients will choose to disable ICD therapies, resulting in fewer shocks (and greater comfort) in the final days of a terminal illness [20].

●Long-term recovery – Patients in all states of health, ranging from those with a terminal-illness to active and healthy patients without an advanced life-threatening illness, may request that they not be resuscitated due to a fear of recovery to an incapacitated state requiring long-term mechanical support. Because an ICD treats potentially lethal arrhythmias quite rapidly, patients may recover to their baseline condition after the device has discharged. However, it should be noted that recurrence of ventricular arrhythmias is high, and their presence may not only be a manifestation but also a driver of worsening cardiac status.

Discontinuing pacemaker therapy in the nonpacemaker-dependent patient — For patients who are not pacemaker-dependent, discontinuing pacemaker therapy (either by reprogramming or removing the device) would not be expected to immediately result in death (algorithm 1). In nearly all cases, the device may be noninvasively reprogrammed such that device removal is not typically required to accomplish withdrawal of therapy.

While potentially controversial to some patients and providers, the discontinuation of a permanent pacemaker (PPM) in patients who are not PPM-dependent is generally considered reasonable if desired by the patient; this decision amounts to withholding rather than withdrawing therapy. Among 658 respondents to a survey regarding the withdrawal of PPM therapy at the end of life, legal professionals (89 percent), medical professionals (87 percent), and patients (79 percent) were consistent in their opinion that withdrawal of PPM therapy in a patient who was not PPM-dependent was appropriate if requested by the patient [21].

Discontinuing pacemaker therapy in the pacemaker-dependent patient — More challenging and controversial is the decision to withdraw PPM therapy in a patient who is PPM-dependent, which would generally be considered a withdrawal of therapy. For patients who are pacemaker-dependent, discontinuing pacemaker therapy (either by reprogramming or removing the device) will almost certainly result in symptomatic bradycardia or asystole (algorithm 1). Clinicians should notify the patient (or the patient's proxy) of the likelihood of loss of consciousness related to bradycardia or asystole and may wish to consider administering additional sedation to the patient at the time the pacemaker is reprogrammed should that decision ultimately be made. The 2018 American College of Cardiology/American Heart Association/Heart Rhythm Society Guidelines support shared decision-making regarding discontinuation of PPM therapy; these guidelines state “patients with decision-making capacity or his/her/their legally defined surrogate have the right to refuse or request withdrawal of pacemaker therapy, even if the patient is pacemaker dependent” [22].

It should be noted that turning off the pacing function of a pacemaker may not lead to the immediate demise of the patient. Many patients have an underlying junctional or ventricular escape rhythm that may sustain life for several hours, days, or even longer. During this time, a patient may have worse quality of life due to syncope or significant lightheadedness. For patients who have cardiac resynchronization therapy (biventricular pacemaker), their underlying heart failure symptoms may also worsen after withdrawal of pacing.

Among the same 658 respondents to the survey discussed above regarding the withdrawal of PPM therapy at the end of life, greater numbers of legal professionals (85 percent) than patients (66 percent) and medical professionals (63 percent) thought that a pacemaker could be turned off in a pacemaker-dependent patient [21]. In a separate survey, an even larger proportion of electrophysiologists (318 of 384 respondents; 83 percent) responded that deactivation of antitachycardia therapies in an ICD was ethically and morally different than discontinuing pacemaker therapy in a pacemaker-dependent patient [23].

However, given the competent patient's ultimate autonomous right to medical decision-making, the patient retains an absolute right to request turning off a PPM in a pacemaker-dependent patient if an informed discussion has taken place between the patient (or proxy) and clinician. In the context of an ethical discussion, regardless of the fact that a patient is pacemaker dependent, the pacemaker still represents an artificial life-sustaining treatment that the patient has the right to refuse at any time [12,18]. Patient autonomy as well as the clinician's ability to withdraw or withhold treatment considered to be futile or otherwise inappropriate are highly important components of any discussion regarding the withdrawal of PPM therapy in a PPM-dependent patient. (See "Ethical issues in palliative care".)

On occasion, the clinician who is asked to withdraw pacemaker therapy in a pacemaker-dependent patient may disagree with the decision to withdraw pacemaker therapy or may have a conscientious objection to performing this function. In this situation, the patient should be referred for another opinion to a clinician with expertise in pacemakers, or a consultation with the hospital ethics committee may be requested.

LOGISTICS OF CIED DEACTIVATION — Important logistic considerations include documentation of the care plan, choice of the appropriate environment for CIED reprogramming, and the appropriate CIED settings.

Discussion and documentation — Prior to any changes in CIED programming, there should be adequate discussion between the patient (or proxy) and clinician regarding the process and expected clinical progression with or without device deactivation, along with documentation of the discussion and the wishes of the patient (or proxy) [24,25]. With the exception of urgent or emergent situations (ie, recurrent ICD shocks delivered to the patient), reprogramming of the CIED, particularly in hospitalized patients, should not occur until an order and/or a note documenting the discussion has been placed in the patient's record.

Choosing the proper location — Choice of the most appropriate location for device deactivation is most relevant for pacemaker-dependent patients, as in most instances these are the only patients likely to experience an abrupt change in clinical status.

●Because of the high likelihood of symptomatic bradycardia or asystole following PPM deactivation in a pacemaker-dependent patient, these patients should have their device deactivated in a setting in which additional sedation can be administered prior to the PPM being reprogrammed. Typically, this occurs in a health care environment (eg, hospital, nursing home) or at home with hospice care.

●Since most non-pacemaker-dependent patients and patients with an ICD typically experience few, if any, immediate consequences following device deactivation, these patients can have their devices reprogrammed anywhere that is feasible for the patient and the clinician.

Device reprogramming — Typically, the reprogramming of the CIED can be handled by the clinician who routinely follows the patient for CIED-related issues, but any clinician who is trained in the management of CIEDs can reprogram the device.

The following are examples of the types of reprogramming:

●For patients with an ICD, tachycardia therapies (ie, antitachycardia pacing and ICD shocks) can be disabled. If pacemaker support is necessary and remains consistent with the goals of care, contemporary ICDs can be programmed to maintain functionality as a pacemaker.

If planned ICD inactivation has not yet been performed, emergent deactivation of antitachycardia therapies, including antitachycardia pacing and shocks, can be accomplished for most devices by placing a magnet over the ICD generator. While the magnet is in place, the ICD will withhold antitachycardia therapy while continuing with pacing functions. When the magnet is removed, the original parameters are restored.

●For patients with a permanent pacemaker (PPM), some devices may be programmed to an OOO mode. Alternatively, the lower rate limit or output may be reprogrammed so that the device is functionally programmed "off."

SUMMARY AND RECOMMENDATIONS

●Discontinuing therapy – Discontinuation of cardiac implantable electronic device (CIED) therapy, including both permanent pacemakers (PPMs) and implantable cardioverter-defibrillators (ICDs), is a complicated issue. Patients at any stage of health or illness should be encouraged to engage in advance care planning to identify their goals of care, specifically addressing CIED management when the patient has an active device. (See "Advance care planning and advance directives" and "Discussing goals of care".)

●Key concepts in palliative care – The utility of late-life ICD therapies will be viewed differently according to the wishes of the patient or proxy. Patients who wish to have all possible efforts at prolonging life will likely desire to maintain ICD therapies and accept the potentially negative side effects (eg, shock-related pain, anxiety, etc). However, for those patients who decline life-prolonging therapies, receiving ICD shocks near the end of life can have a profoundly negative impact on quality of life and may also impact family and friends. (See 'Key concepts in palliative care' above.)

●Indications for discontinuation – A discussion regarding the discontinuation of CIED therapy should typically arise when the goals of care have changed or a CIED complication has occurred. Patients with capacity (or the patient's legally designated surrogate) can request discontinuation of any medical or device treatment, including therapies such as pacemaker treatment in a pacemaker-dependent patient. (See 'Indications for discontinuing CIED therapy' above.)

●What the patient and family should expect following CIED discontinuation varies depending on the device and clinical scenario (algorithm 1). (See 'What to expect after device discontinuation' above.)

•For most patients with an ICD, discontinuing antitachycardia therapies (including antitachycardia pacing and shocks) by reprogramming the device will not have any immediate impact on comfort or quality of life. The only exception would be in a patient who is receiving, or has recently received, one or more ICD shocks. In this case, quality of life may improve by reducing painful shocks, but a recurrent ventricular arrhythmia would be untreated and potentially fatal.

•For patients who are not pacemaker-dependent, discontinuing pacemaker therapy (either by reprogramming or removing the device) would not be expected to immediately result in death or have an immediate impact on comfort or quality of life.

•For patients who are pacemaker-dependent, discontinuing pacemaker therapy (either by reprogramming or removing the device) will almost certainly result in symptomatic bradycardia or asystole. Clinicians should notify the patient (or the patient's proxy) of the likelihood of loss of consciousness related to bradycardia or asystole and may wish to consider administering additional sedation to the patient at the time the pacemaker is reprogrammed.

●Logistics of deactivation – Following discussion between the patient (and/or the patient's healthcare proxy) and the clinician and decision-making regarding deactivation of cardiac implantable electronic device (CIED) therapies, the CIED can usually be reprogrammed to provide or withhold the desired therapies. Important logistic considerations including documentation of the care plan, choice of the appropriate setting for CIED reprogramming, and the appropriate environs in which to make the programming changes. (See 'Logistics of CIED deactivation' above.)

ACKNOWLEDGMENT — The UpToDate editorial staff acknowledges Ann Garlitski, MD, who contributed to earlier versions of this topic review.