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INTRODUCTION — Discontinuation of cardiac implantable electronic device (CIED) therapy, which encompasses both pacemakers and defibrillators, is a complicated issue. For many patients, these are life-sustaining therapies. In 2010, the Heart Rhythm Society (HRS) released an expert consensus statement on the management of CIEDs in patients nearing the end of life or requesting withdrawal of therapy . This statement outlines the 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. Additional discussion of the ethical issues involved in palliative care is presented separately. (See "Ethical issues in palliative care".)
FREQUENCY OF LATE-LIFE ICD THERAPIES — Available data to guide providers in counseling patients have been obtained from studies of implantable cardioverter-defibrillator (ICD) interrogations and interviews with families.
●Interviewing 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 .
●A retrospective chart review of 98 patients with ICDs from the Multicenter Automated Defibrillator Implantation Trial II who later died found that 15 percent chose to have their ICDs deactivated. Ten patients whose ICDs remained activated received shocks during the 24 hours before death .
●In a prospective analysis of 125 explanted ICDs, 35 percent of the patients were noted to have ventricular tachyarrhythmias in the last hour of their lives. Twenty-four percent had an arrhythmic storm, and 31 percent received shock treatment during the last 24 hours. However, arrhythmic death was the primary cause of death in only 13 percent. 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 .
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 have chosen to withdraw ICD 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.
INDICATIONS FOR DISCONTINUING CIED THERAPY — The discussion around discontinuation of cardiac implantable electronic device (CIED) therapy is different depending on whether the device in question is a permanent pacemaker (PPM) or an implantable cardioverter-defibrillator (ICD). 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 an example, 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.
●A belief that the device is no longer necessary (perceived reduction in risk) – In the case of an ICD, it may be because the device has never delivered a therapy and/or there has been an improvement in the patient's cardiac condition. In the case of a PPM in a non-PPM dependent patient, it may be because the device is infrequently, if ever, required as the patient has an adequate heart rate and rhythm.
●A CIED complication has occurred – Most commonly due to infection or device component malfunction necessitating CIED removal.
Patient preference — 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. No patient is committed to therapy that he or she no longer wishes to receive . In addition, it is not necessary for patients to be terminally ill to make these requests. (See "Ethical issues in palliative care".)
One challenge encountered by clinicians, patients, and loved ones with the withdrawal of any specific therapy which is perceived as life-sustaining is that the process by which death occurs following the discontinuation of specific therapies, and the associated emotional impact, can vary dramatically. For example, discontinuation of a therapy such as hemodialysis may lead to death in days to weeks, while the deactivation of a pacemaker in a pacemaker-dependent patient may lead to immediate loss of consciousness and death. Ultimately, a patient-directed (or proxy-directed) request for withdrawal of care that potentially has an immediate impact on the timing of death, such as withdrawal of pacemaker therapy in a pacemaker-dependent patient, is ethically sound and should be distinguished from an act of euthanasia. As with the decisions regarding any medical intervention, communication between the care providers, patients, and loved ones at all steps of the process is critical, beginning with capacity assessment to make the decision; exploration of values, goals and reasons for the decision; and for aligning expectations about the expected outcomes of the treatment decision. (See "Euthanasia and physician-assisted death".)
Perceived reduction in risk — Patients (or their healthcare proxies) may request that a CIED be removed or disabled either because of a perceived lack of need for the device or because of a change in the goals of care. There is little experience or data to define which patients, if any, who had an appropriate indication for ICD implantation could safely have an ICD disabled or removed due to a reduction in sudden cardiac death (SCD) risk. Similarly, there is little data to define which non-pacemaker dependent patients who had an appropriate indication for PPM implantation could safely have the PPM removed due to a perceived reduction in risk of future conduction abnormalities requiring pacing.
Device complications necessitating CIED removal — It is occasionally necessary to remove a CIED system, the generator, and accompanying lead(s) due to a complication, most commonly structural failure of a CIED component such as lead fracture, or CIED system infection. When a pacemaker is removed, the indication for pacing versus the risks of reimplantation has to 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 "Implantable cardioverter-defibrillators: Complications" and "Infections involving cardiac implantable electronic devices".)
Removal or replacement of the generator (which includes the battery) is relatively straightforward and is usually done in the electrophysiology laboratory. In contrast, lead extraction typically requires specialized equipment, and, due to the risk of major cardiac and vascular injury, it is often performed in an operating room. (See "Cardiac implantable electronic device lead removal".)
DISCONTINUATION OF ICD THERAPY — When discontinuation of implantable cardioverter-defibrillator (ICD) therapy is requested because of a change in the goals of care or a perceived reduction in risk, the role of the clinician is to fully explain the implications of the decision. Some issues that may be important include:
●Long-term recovery – Some active and healthy patients without an advanced life-threatening illness 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 very rapidly, however, patients usually recover to their baseline condition after the device has discharged.
●Shock-related pain and anxiety – Among patients with a terminal illness and a poor short-term 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. 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 .
●Perceived reduction in risk — Data from trials of ICD therapy for primary prevention have shown that many patients first experience sudden cardiac death (SCD) several years after a myocardial infarction . Thus, a prolonged period without an arrhythmic event should not be interpreted as evidence that the patient is no longer at risk. (See "Primary prevention of sudden cardiac death in heart failure and cardiomyopathy".)
DISCONTINUATION OF PPM THERAPY
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".)
Discontinuing pacemaker therapy in the nonpacemaker-dependent patient — 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-depending was appropriate if requested by the 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 and, except for any associated discomfort in the event of a device removal, would not generally be associated with any discomfort.
Discontinuing pacemaker therapy in the pacemaker-dependent patient — More challenging and potentially controversial is the decision to withdraw PPM therapy in a patient who is PPM-dependent, which would generally be considered a withdrawal of therapy. Among the same 658 respondents to the survey discussed above regarding the withdrawal of PPM therapy at the end of life, more than one-third of medical professionals (37 percent) and patients (34 percent) but only 15 percent of legal professionals thought that a pacemaker should not be turned off in a pacemaker-dependent patient because doing so would be considered clinician-assisted death . 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 .
However, given the competent patient's ultimate autonomous right to medical decision making, turning off a PPM in a pacemaker-dependent patient is also reasonable 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 [1,5]. Patient autonomy as well as the clinician's ability to withdraw or withhold care 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".)
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 off.
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 cardiac implantable electronic device (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 data [10-12].
●In a retrospective, single-center study of the prevalence of an advanced directive among 420 patients who underwent implantable cardioverter-defibrillator (ICD) implantation, only 28 percent had an advance directive in place at the time of ICD implantation, with only two specifically addressing the issue of ICD deactivation at end of life .
●In a single-center telephone survey of 278 patients with an ICD, more than half of the patients had executed an advance directive, but only three (1 percent) had included a plan for the management of the ICD .
●In a nationwide cross-sectional 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 .
Clinicians and advance care planning — Advance care plans should be an integral part of all clinician interactions with 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. As examples:
●In a nationwide survey of US clinicians, one-third of internist respondents and two-thirds of electrophysiologist respondents believed their patients already knew they could deactivate the shocking function of an ICD . 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 . Clinicians who believe that patients know the options for device management at the end of life may be less likely to have deactivation conversations .
●In a survey of 294 patients in the Netherlands, 68 percent believed that it is possible to turn the ICD off . In contrast, in a smaller study in the US of 54 patients, only 3 percent recalled receiving information about this option when providing consent for implantation, and 38 percent became aware at a later stage .
●In one single-center, retrospective chart review of 150 patients who underwent device deactivation at a tertiary care center, 42 percent of patients who had their device deactivated received 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 . A separate study revealed that only 10 percent of hospice providers have a device deactivation policy .
When patient and clinician beliefs differ — 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. Heart Rhythm 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 [1,18]. 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/inadvisable therapies".)
LOGISTICS OF CIED DEACTIVATION — Once the decision has been made to deactivate cardiac implantable electronic device (CIED) therapies following a face-to-face meeting between the patient (and/or the patient's health care proxy) and the clinician, the CIED can usually be reprogrammed electronically to provide or withhold the desired therapies. There are only rare circumstances (ie, CIED infection or significant CIED malfunction) which would require the CIED to be physically removed from the patient.
Prior to any changes in CIED programming, there should be adequate documentation of the patient (or proxy) and clinician discussion, and of the wishes of the patient (or proxy). With the exception of urgent or emergent situations (ie, incessant implantable cardioverter-defibrillator [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.
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 which can occur:
●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. While the magnet is in place, the ICD will withhold antitachycardia therapy. If the magnet is removed, then 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."
The decision-making regarding CIED reprogramming should take place as part of a face-to-face discussion between the patient (or proxy) and the clinician prior to making a decision of this magnitude.
SUMMARY AND RECOMMENDATIONS
●In general, a discussion regarding the discontinuation of cardiac implantable electronic device (CIED) therapy typically arises when there is a belief that the device is no longer necessary, the goals of care have changed, or a CIED complication has occurred. (See 'Indications for discontinuing CIED therapy' above.)
●Among patients with a terminal illness and a poor short-term prognosis, the pain from an implantable cardioverter-defibrillator (ICD) shock, or the anxiety of potentially receiving an ICD shock, can be contrary to a primary goal of maintaining the patient's comfort. 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 preferences for end-of-life care. (See 'Discontinuation of ICD therapy' above.)
●In the context of an ethical discussion, regardless of the fact that a patient is pacemaker dependent, a permanent pacemaker (PPM) still represents an artificial life-sustaining treatment that the patient has the right to refuse at any time. Patient autonomy as well as the clinician's ability to withdraw or withhold care 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 'Discontinuation of PPM therapy' above.)
●Patients with an implanted CIED at any stage of health or illness should be encouraged to complete advance directives, which should also specifically address CIED management. Advance directives 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' above.)
●Once the decision has been made to deactivate CIED therapies following a face-to-face meeting between the patient (and/or the patient's health care proxy) and the clinician, the CIED can usually be reprogrammed electronically to provide or withhold the desired therapies. There are only rare circumstances (ie, CIED infection or significant CIED malfunction) which would require the CIED to be physically removed from the patient. (See 'Logistics of CIED deactivation' above.)
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- Sherazi S, McNitt S, Aktas MK, et al. End-of-life care in patients with implantable cardioverter defibrillators: a MADIT-II substudy. Pacing Clin Electrophysiol 2013; 36:1273.
- Kinch Westerdahl A, Sjöblom J, Mattiasson AC, et al. Implantable cardioverter-defibrillator therapy before death: high risk for painful shocks at end of life. Circulation 2014; 129:422.
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