Basic ethical principles applicable to patient care include respect for patient autonomy (the duty to respect patients and their rights of self-determination), beneficence (the duty to promote patient interests), nonmaleficence (the duty to prevent or not harm patients), and justice, referring in part to the duty to treat patients and distribute health care resources fairly.⁵ Informed consent, the most important legal doctrine in the clinician-patient relationship, derives from the ethical principle of respect for persons; autonomy is maximized when patients understand the nature of their diagnoses and treatment options and participate in decisions about their care. The rationale for informed consent is simple: it is the patient’s body and life—the patient must live with the consequences of the treatment—and thus the patient has the most at stake in the decision.⁶ Clinicians are ethically and legally obligated to ensure that patients are informed about their diagnoses and treatment options.^7,⁸

The U.S. courts have ruled that the right to make decisions about medical treatments is both a common law (derived from court decisions) right based on bodily integrity and self-determination and a constitutional right based on privacy and liberty.⁶ A corollary to informed consent is informed refusal. A patient has the right to refuse any treatment, even if the treatment prolongs life, or death would follow a decision not to use it. A patient also has the right to refuse a previously consented treatment if the treatment no longer meets the patient’s health care goals, if those goals have changed (e.g., from prolonging life to minimizing discomforts), or if the perceived burdens of the patient’s illness (e.g., quality of life) and ongoing treatment outweigh the perceived benefits of ongoing treatment.^6,⁹^–¹¹ Honoring these decisions is an integral part of patient-centered care. If a clinician initiates or continues a treatment that a patient has refused, then ethically and legally the clinician is committing “battery,” regardless of the clinician’s intent.⁷ Finally, granting requests to withdraw life-sustaining treatments from patients who do not want them, is respecting a right to be left alone and to die naturally of the underlying disease, a legally protected right based on the right to privacy. This has been phrased as “a right to decide how to live the rest of one’s life.”

A patient’s right to withdraw unwanted treatment has been consistently upheld by U.S. courts (Table 35-1). In the In re Quinlan case, the New Jersey Supreme Court ruled that the patient had both common law and constitutional rights to refuse continued ventilator support, even though her clinicians believed she would die without it.¹² In the Cruzan v. Director, Missouri Department of Health case, which involved a feeding tube, the U.S. Supreme Court ruled the same way; that is, patients have the right to refuse life-sustaining treatments. The Court also ruled that a feeding tube was a medical treatment and that it did not have unique status.¹³ Whether a feeding tube had unique status was raised again during the Terri Schiavo case. The courts again ruled adult patients have a constitutional right to refuse any treatment, including life-sustaining treatments, and that there is no legal difference between withdrawing an ongoing treatment and not starting it in the first place.¹⁴

TABLE 35-1 Landmark Legal Cases Confirming the Right to Withhold or Withdraw Life-Sustaining Therapies

In none of these cases did the courts distinguish between types of life-sustaining treatments. The law applies to the person, and informed consent is a right of the patient—it is not specific to any one medical intervention.^6,¹⁴^–¹⁶ Thus, even though the Supreme Court has not specifically commented on the question of pacemaker or ICD deactivation, because CIEDs deliver life-sustaining therapies, discontinuation of these therapies is clearly addressed by the previous Supreme Court precedents upholding the right to discontinue life-sustaining treatment.

In addition, these rights extend to patients who lack decision-making capacity, through previously expressed statements (e.g., advance directive) and surrogate decision makers.^11,^14,¹⁷ When patients lack decision-making capacity because of medical illness (e.g., dementia), clinicians must rely on surrogates to make decisions. If the patient has an advance directive (AD) that identifies a surrogate, the patient’s choice of surrogate should be respected⁷ (see later). In the absence of an AD, clinicians must identify the legally recognized appropriate surrogate. The ideal surrogate is one who best understands the patient’s health care–related goals and preferences. Many U.S. states, however, specify by law a hierarchy of surrogate decision makers (e.g., spouse, followed by adult child, and so on). When making decisions, a surrogate should adhere with the instructions in the patient’s AD (if one exists) and base decisions on the patient’s—not the surrogate’s—values and preferences if known (i.e., the “substituted judgment” standard).¹⁸

Although both the law and ethics are clear—a patient has the right to refuse and request the withdrawal of CIED therapies regardless of whether he or she is terminally or irreversibly ill, and regardless of whether the therapies prolong life and death would follow a decision not to use them ⁷—some clinicians who care for patients with CIEDs see a moral distinction between deactivating an ICD and deactivating a pacemaker, particularly in a dependent patient.² However, while some have questioned whether pacemaker deactivation is similar to assisted suicide or euthanasia,² there are several key differences between withdrawal of CIED therapies and assisted suicide. First is the issue of clinician intent. When a physician complies with a patient’s request to deactivate a device, the physician’s intent is to remove a treatment that is perceived by the patient as burdensome or is simply unwanted. Although this may have the effect of allowing the patient to die of an underlying disease,^12,^16,¹⁹ hastening death should not be the clinician’s primary intent.^7,^10,²⁰ In contrast, in assisted suicide, the patient intentionally terminates his own life using a lethal method provided or prescribed by a clinician. In euthanasia, the physician directly and intentionally terminates the patient’s life (e.g., lethal injection). The second point differentiating withdrawal of an unwanted therapy from assisted suicide and euthanasia lies in the cause of death. In assisted suicide or euthanasia, death is caused by the intervention provided, prescribed, or administered by the clinician. In contrast, when a patient dies after a treatment is refused or withdrawn, the cause of death is the underlying disease. There are clearly nuances to this issue. For example, a pacemaker-dependent patient with depression may request deactivation of the pacemaker because life itself is burdensome. In such situations it is important to determine the decision-making capacity of the patient.

The U.S. Supreme Court decisions have made a clear distinction between withdrawing life-sustaining treatments and assisted suicide and euthanasia. In the case of Vacco v. Quill,²¹ Chief Justice Rehnquist wrote:

The distinction comports with fundamental legal principles of causation and intent. First, when a patient refuses life-sustaining medical treatment, he dies from an underlying fatal disease or pathology; but if a patient ingests lethal medication prescribed by a physician, he is killed by that medication. … [In Cruzan] our assumption of a right to refuse treatment was grounded not. … on the proposition that patients have a … right to hasten death, but on well established, traditional rights to bodily integrity and freedom from unwanted touching.

The Court ruled that all patients have a constitutional right to refuse treatment, but no one has a constitutional right to assisted suicide or euthanasia. In another case,²² the Court ruled that clinicians can legally (and should, from an ethical perspective) provide patients with whatever treatments needed to alleviate suffering, such as morphine, even if the treatments might hasten death. Criminality is determined by the clinician’s intent.

Legal precedent does not distinguish between types of unwanted therapies, but there are aspects of pacing therapies in a dependent patient that some clinicians have found problematic. For example, ICD therapies are intermittent whereas pacemaker therapy in a pacemaker-dependent patient is continuous; death might not occur immediately after ICD deactivation, whereas death might occur quickly after pacemaker deactivation. However, widespread agreement exists that withdrawing other continuous life-sustaining treatments, such as mechanical ventilation, is ethically and legally permissible. Similarly, clinicians may consider duration of therapy as morally important when considering requests to withdraw life-sustaining treatments. However, withdrawal of other long-term life-sustaining treatments, such as hemodialysis and artificial hydration and nutrition, is well accepted.²³ Another concern expressed about withdrawing CIED therapies is that the devices, unlike other life-sustaining treatment, are completely internal. However, the ethical and legal principles involved in the right to refuse treatment are not based on the location of the therapy. Thus, the fact that pacemakers provide therapy that is continuous, internal, and of long duration does not detract from the permissibility of carrying out requests to withdraw this therapy from patients who no longer want it.

Some disagreement exists regarding whether a pacemaker is a substitutive therapy, one that substitutes for a pathologically lost function, or a replacement therapy, one that replaces a pathologically lost function. There is agreement that substitutive life-sustaining treatments, such as hemodialysis for kidney failure or a ventilator for respiratory failure, can be withdrawn. A replacement therapy, such as kidney transplantation for kidney failure, literally becomes “part of the patient” and provides the lost function in the same fashion as did the patient’s own body. Replacement therapies also respond to physiologic changes in the host and are independent of external energy sources and control of an expert. Removing or withdrawing a replacement life-sustaining treatment has been characterized as “euthanasia.”²³ CIED therapies, including pacemaker support in a pacemaker-dependent patient, lack the features of replacement therapies and therefore are most often characterized as “substitutive,”²⁴ although this distinction has been questioned.¹⁹ A transplanted kidney, however, has all the features of a replacement therapy. Most would regard carrying out a request to deactivate a pacemaker in a terminally-ill patient as far less morally problematic than carrying out a request to remove a transplanted kidney in the same patient. Deactivating a pacemaker is noninvasive and does not introduce a new pathology (although it may precipitate heart failure symptoms). Removing a kidney, however, is invasive and introduces a new pathology (i.e., a wound).

Ethical Principles Underlying, the Decision-Making Process

Who decides about device deactivation? Ethically and legally, competent patients (or their surrogates) have authority to make decisions. Patients’ decisions have priority over clinicians’ decisions. Because a clinician regards a patient’s decision as “wrong” does not mean the decision is irrational. What criteria should go into making the decision? The patient and physician together must determine a treatment’s effectiveness and the benefits and burdens in the context of the patient’s illness and quality of life. A treatment’s effectiveness is its ability to alter the natural history of a disease. CIEDs are effective, for example, in bypassing life-threatening cardiac conduction abnormalities or treating fatal arrhythmias. However, treatment effectiveness is not the same as treatment benefits and burdens. Benefit is determined by the patient: the patient’s assessment of the treatment’s value. Burden is also determined by the patient: the patient’s assessment of the existing and potential discomforts, costs, and inconveniences associated with the illness and its treatment as the patient perceives it.⁹ Each patient is unique and weighs such benefits and burdens in relation to their own values, preferences, and health care–related goals. What one patient perceives as beneficial and nonburdensome may be viewed by another patient as nonbeneficial and burdensome. Because benefit and burden are determined by the patient, a patient may decide the burden of CIED therapy outweighs the benefit even if he is not terminally ill.

Although patients are the ultimate decision makers, conflicts between caregivers and patients can arise, often when there is misunderstanding among patients and clinicians on goals of care. For example, a clinician may view ongoing CIED therapy in a terminally ill patient as effective, beneficial, and nonburdensome. The patient (or surrogate), however, may strongly disagree. Multidisciplinary care conferences, ethics consultation, and palliative care consultation can be very helpful in resolving these conflicts, especially in clarifying goals of care, establishing the permissibility of withdrawing CIED therapies (and contingency plans if a decision is made not to withdraw CIED therapies), and formulating care plans.

Ethics consultation is not required before device deactivation. However, this may be helpful in ambiguous situations such as conflict between members of a family or disagreement between members of the health care team caring for a patient, or caregivers may find that additional support is needed when pursuing a particular treatment course.²⁵ If the decision-making capacity of the patient is in question, or if there are suspicions of coercion, ethics consultation can also be helpful. The Joint Commission requires that health care institutions have processes for addressing ethical concerns that arise in the care of patients.²⁶ Case 3, describing disagreement among both family and caregivers, and case 4, an ambiguous case illustrating possible coercion, illustrate examples in which ethics consultation may be helpful:

Case 3

An 81-year-old woman with complete atrioventricular (AV) block and no structural heart disease received a permanent DDD pacemaker 18 years ago; the generator was replaced 8 years ago. She is now in a nursing home with progressive dementia. Her son calls and asks if her cardiologist can come and turn off her pacemaker. The cardiologist feels uncomfortable honoring this wish, although the pacemaker nurse who would actually perform the deactivation does not. Before any decisions are made, a daughter calls saying her mother would never want her pacemaker deactivated.

Case 4

An 85-year-old woman had received a pacemaker for congestive heart block 2 years ago. Although her religious group does not believe in implanted devices, she agreed to the device in the hospital. Now, however, she wants to enter an assisted-living/nursing facility run by her group, but they will not accept her with an active pacemaker. She requests deactivation.

Advance care planning can often prevent ethical dilemmas at the end of life. In this process, which promotes patient autonomy, a patient identifies his or her values, preferences, and goals regarding future health care (e.g., at the end of life) and a surrogate decision maker in the event the patient loses decision-making capacity.⁷ Ideally, the patient should discuss values and preferences with care providers and potential surrogate decision makers, who should document them in the patient’s medical record, and the patient should complete an advance directive. There are two forms of ADs: the durable power of attorney for health care and the living will. The durable power of attorney for health care allows the patient to specify a surrogate in the event the patient loses decision-making capacity. The living will allows the patient to list specific health care–related values, goals, and preferences. From an ethics standpoint, clinicians should view the AD as an extension of the autonomous person and therefore should respect the values, goals, and preferences listed in the AD. From a legal standpoint, all 50 U.S. states recognize ADs as an extension of the autonomous person. In fact, the Patient Self-Determination Act, passed by Congress in 1990 in response to the Cruzan decision, requires that health care institutions that participate in Medicare and Medicaid programs ask patients whether they have an AD, inform patients of their rights to accept or refuse medical treatments and to create and execute an AD, and to incorporate ADs into patients’ medical records.²⁷

Rights and Responsibilities of Clinicians for Whom Deactivation Is Counter to Their Personal Beliefs

Regardless of the ethical and legal permissibility of carrying out requests to withdraw CIED therapies from patients who have (or their surrogate has) made this decision, clinicians—like patients—are moral agents whose personal values and beliefs may lead them to prefer not to participate in device deactivation. A recent survey found that about 10% of clinicians who care for patients with CIEDs view pacemaker deactivation in a pacemaker-dependent patient as a form of assisted suicide or euthanasia.² Others object to pacemaker deactivation because they believe pacemaker therapy does not prolong the dying process or cause physical discomfort (unlike ICD shocks) and that pacemaker deactivation may cause discomfort (e.g., worsened heart failure symptoms).²⁸ These burdens are ultimately determined by the patient, but clinicians and others, including industry employed allied professionals, should not be compelled to carry out device deactivations if they view the procedure as inconsistent with their personal values.^8,²⁸ Under these circumstances, the clinician should inform the patient of the clinician’s preference not to perform CIED deactivation. However, as described in the American Medical Association (AMA) Code of Medical Ethics,²⁹ the clinician should not impose his or her values on or abandon the patient and should ensure that they do not cause the patient emotional distress.³⁰ Instead, the clinician and patient should work to achieve a mutually agreed-upon care plan. If such a plan cannot be achieved, the primary clinician should involve a second clinician who is willing to comanage the patient and provide legally permissible care and procedures, including CIED deactivation.^28,²⁹ It is important for the health care team to recognize and address any conflicts within the team. It is also the responsibility of the institution to ensure that services that are legal and that may be requested by the patient are available.

Preventing Both Ethical Dilemmas and Painful Shocks at End of Life: Importance of Early, Proactive Communication

“It hadn’t occurred to me to turn it off until [the cardiology fellow said] you could turn these things off and I’m like, ‘oh, okay.’ I mean it wasn’t something that I had ever encountered, and it crossed my mind on a technical level, but not really, ‘Oh, I should have this conversation.’ ”

ICD patient³¹

Case 5

A 74-year-old woman had an ICD inserted 5 years ago after her first episode of ventricular tachycardia. As her heart disease progressed, the episodes of ventricular tachycardia increased. While at home one afternoon, her defibrillator shocked her several times, and she was admitted to the hospital. She went in and out of consciousness and was hemodynamically unstable for the next 72 hours. After a long family meeting, her family and cardiologist decided that no further aggressive treatments would be continued and that her care would focus on comfort. During the night, her ICD shocked her 10 times, while her nurse tried desperately to contact someone to turn off her ICD. Finally, at 3 am, the cardiologist came in and deactivated the ICD. She died 2 hours later.¹⁷

Timely and effective communication among patients, families, and health care providers is essential to informed consent and to prevent situations such as described in Case 5. Effective communication includes determining the patient’s goals of care, helping the patients weigh the benefits and burdens of device therapy as his clinical situation changes, clarifying the consequences of deactivation, and discussing potential alternative treatments, as well as encouraging the patient to complete an AD. Clinicians must take a proactive role in discussions about the option of deactivation in the context of the patient’s goals for care. These conversations should continue over the course of the patient’s illness. As illness progresses, patient preferences for outcomes and the level of burden acceptable to a patient may change.^32,³³ Advanced care planning conversations improve outcomes for both patients and their families,³⁴ as patients with ICDs who engage in advance care planning are less likely to experience shocks while dying because ICD deactivation has occurred.³⁵ Studies show that patients and families desire conversations about end-of-life care.³⁶^–³⁸

Few patients with CIEDs discuss device deactivation with their clinicians or know that device deactivation is an available option.^1,^3,³¹ Even though many patients with CIEDs have ADs, very few of them mention the device specifically in their ADs.³⁹ Clinicians must take a proactive role in initiating discussions about the option of deactivation, from the time of implantation throughout a patient’s life as the clinical situation changes (Table 35-2). The complexity of these conversations, however, is evident in data from physicians demonstrating that although they believe they should engage in these types of conversations with patients, they rarely do.^1,^3,³¹

TABLE 35-2 Physician-Initiated Discussions with Implant Patients

Timing of Conversation	Points to Discuss	Helpful Phrases to Consider
Before implantation	Clear discussion of benefits and burdens of device. Brief discussion of potential future limitations or burdensome aspects of device therapy. Encourage patients to have some form of advance directive. Inform of option to deactivate device in the future.	“It seems clear at this point that this device is in your best interest, but you should know at some point if you become very ill from your heart disease or another process you develop in the future, the burden of this device may outweigh its benefit. While that point is hopefully a long way off, you should know that turning off your defibrillator is an option. ”
After an episode of increased or repeated firings from an ICD	Discussion of possible alternatives, including adjusting medications, adjusting device settings, and cardiac procedures to reduce future shocks.	“I know that your device caused you some recent discomfort and that you were quite distressed. I want to work with you to see if we can adjust the settings to assure that the device continues to work in the appropriate manner. If we can’t get you to that point, then we may want to consider turning it off altogether, but let’s try some adjustments first.”
Progression of cardiac disease, including repeated hospitalizations for heart failure and/or arrhythmias	Reevaluation of benefits and burdens of device. Assessment of functional status, quality of life, and symptoms. Referral to palliative and supportive care services.	“It appears as though your heart disease is worsening. We should really talk about your thoughts and questions about your illness at this point and see if your goals have changed at all.”
When patient/surrogate chooses a “do not resuscitate” (DNR) order*	Reevaluation of benefits and burdens of device. Exploration of patient’s understanding of device and how patient conceptualizes it with regard to external defibrillation. Referral to palliative care or supportive services.	“Now that we’ve established that you would not want resuscitation in the event your heart were to go into an abnormal pattern of beating, we should reconsider the role of your device. In many ways it is also a form of resuscitation. Tell me your understanding of the device, and let’s talk about how it fits into the larger goals for your medical care at this point.”
Patients at end of life	Reevaluation of benefits and burdens of device. Discussion of option of deactivation addressed with all patients, although deactivation not required.	“I think at this point we need to reconsider your [device]. Given how advanced your disease is, we need to discuss whether it makes sense to keep it active. I know this may be upsetting to talk about, but can you tell me your thoughts at this point?”

* Patients may choose to forego intubation, CPR, and external defibrillation while at the same time deciding to keep the defibrillation function of their ICD active. A patient’s choice to be “DNR” may or may not be concomitant with a decision to withdraw CIED therapy, as resuscitation interventions and the ICD each carries its own benefits and burdens.

Modified from Lampert R, Hayes DL, Annas GJ, et al: Heart Rhythm Society Expert Consensus Statement on the management of cardiovascular implantable electronic devices (CIEDs) in patients nearing end of life or requesting withdrawal of therapy. Heart Rhythm 7:1008-1026, 2010.

Discussion of Device Deactivation with Overall Goals of Care

Communication techniques used to discuss the role of the device need to move from treatment-directed conversations to goal-directed conversations. In other words, asking patients and their families, “Do you want the device to remain on?” is often as misleading and misunderstood by them as the question, “Do you want everything done?” Without a better understanding of their current state of health and the role of the cardiac device, patients cannot make fully informed decisions.

Table 35-3 outlines the steps needed for goal-directed communication and some useful phrases to begin conversations at each point. These conversations should include a discussion of quality of life, functional status, perceptions of dignity, and both current and potential future symptoms, because each of these elements can influence how patients set goals for their health care. Step 2 is particularly important, because data shows that some patients with ICDs do not understand the role the device plays in their health, particularly in terms of care at the end of life.⁴⁰ The goal is not to overburden patients with decisions, but to determine an overall set of guiding principles by which clinicians can help patients make decisions. These conversations should follow the model of “shared decision making” in which clinicians work together with patients and families to ensure that patients understand, in the context of their illness, the benefits and burdens of a particular treatment and the potential outcomes that may result from its continued use or discontinuation.⁴¹

TABLE 35-3 Steps for Communicating with Patients and Families about Goals of Care with CIEDs

Step	Sample Phrase to Begin Conversation
1. Determine what patients/families know about their illness.	“What do you understand about your health and what is occurring in terms of your illness?”
2. Determine what the patient and family know about the role that the device plays in their health both now and in the future.	“What do you understand the role of the [CIED] to be in your health now?
3. Determine what additional information the patient and family want to know about the patient’s illness.	“What else would be helpful for you to know about your illness or the role the [CIED] plays within it?
4. Correct or clarify any misunderstandings about the current illness and possible outcomes, including the role of the device.	“I think you have a pretty good understanding of what is happening in terms of your health, but there are a few things I would like to clarify with you.”
5. Determine the patient and family’s overall goals of care and desired outcomes.	“Given what we’ve discussed about your health and the potential likely outcomes of your illness, tell me what you want from your health care at this point.” Note: Sometimes patients and families may need more guidance at this point, so some potential guiding language might be: “At this point some patients tell me they want to live as long as possible, regardless of the outcome, whereas other patients tell me that the goal is to be as comfortable as long as possible while also being able to interact with their family. Do you have a sense of what you want at this point?”
6. Using the stated goals as a guide, work to tailor treatments, and in this case the management of the cardiac device, to those goals.	Phrases to be used here depend on the goals as set by the patient and family. For a patient who states that her desired goal is to live as comfortably as possible for whatever remaining time she has left: “Given what you’ve said about assuring that you are as comfortable as possible it might make sense to deactivate the shocking function of your ICD. What do you think about that?” or For a patient who states he wants all life-sustaining treatments to be continued, an appropriate response might be, “In that case, perhaps leaving the antiarrhythmia function of the device active would best be in line with your goals. However, you should understand that this may cause you and your family discomfort at the end of life. We can make a decision at a future point in time about turning the device off. Tell me your thoughts about this.”

CIEDs, Cardiac implantable electronic devices.

Table adapted from references 1, 35, 38, 48, and 49.

Clinicians must also recognize that while the ultimate power for decision making rests with patients, they may be influenced by factors such as family, culture, or religion. Likewise, although cost should not play a role in the ways that clinicians counsel patients and families, cost does influence the way that some patients make decisions. Studies of patients with serious illness note that many families often lose their savings as well as a major source of income from either the illness itself or from other family members having to care for the patient.^41,⁴² In addition, many of these factors may influence a patient to cede the power of decision making to other individuals.

Benefits/Burdens of Ongoing Device Therapy; Consequences/Uncertainties of Deactivation

An important role for the clinician is to provide factual information concerning the beneficial and negative effects of continuing device therapy. The patient can then assess how the benefits and burdens of continued therapy fit with ongoing health care goals. It is also vital for both the health care provider and the patient to have an accurate understanding of the expected consequences of device deactivation. Although in some cases (e.g., patients who are 100% pacemaker dependent) this may be relatively straightforward, in many situations it will be difficult to predict a patient’s clinical course after deactivation. The timing and lethality of tachyarrhythmias are unpredictable, and bradyarrhythmias may variously manifest with syncope, heart failure, angina, fatigue, or sudden death. Consultation with a clinical electrophysiologist may help clarify the clinical picture, although significant uncertainty will remain in many cases. Case 6 illustrates the importance of communication with the family regarding the unpredictability of consequences of deactivation of ICD therapies:

Case 6

An 80-year-old man with dilated cardiomyopathy in rural Florida received a biventricular ICD for left bundle branch block (LBBB) and congestive heart failure (CHF). He later developed progressive CHF and became bedbound and hypotensive. He had no history of ventricular arrhythmias treated with shocks or antitachycardia pacing (ATP). His daughter asks if the ICD can be turned off immediately. An industry-employed allied professional (IEAP) went to the home and deactivated ATP therapies. The daughter then became angry toward both the IEAP and electrophysiology (EP) team when her father did not die immediately, stating she had a plane to catch to return home.

Once a patient’s goals of care are determined, knowledge about a specific device, whether pacemaker, ICD, or cardiac resynchronization therapy (CRT) device, is essential to determining how to change its settings consistent with the patients’ goals regarding survival and quality of life. Pacemaker and CRT therapy are indicated for the amelioration of symptoms caused by bradycardia and heart failure, respectively.⁴³ For patients who have no underlying intrinsic rhythm (“pacemaker dependent”), pacing also provides life-sustaining therapy. Pacemaker dependence, however, can vary over time.⁴⁴ In a pacemaker-dependent patient, death may follow immediately after cessation of pacing therapy. If the patient is not pacemaker dependent, the dying process is unpredictable, and patients need to be assessed closely for symptoms of distress. Deactivation of pacing therapy may result in symptoms that worsen the quality of life (QOL) of a patient who is not pacemaker dependent, but appropriate symptom control in this group can be used effectively to ensure comfort.⁴⁴ If CRT improves heart failure or reduces arrhythmia burden, discontinuation may impact survival as well as symptoms and QOL. However, ICDs do not improve symptoms, and shocks from an ICD have added to patient and family suffering when the device has fired at the end of life.^1,^17,²⁸ Deactivation of ICD shock therapy may thus improve QOL in such patients by eliminating the pain and emotional distress associated with the delivery of noxious ICD discharges. Elimination of defibrillation therapy is less likely to result in immediate death unless the patient is experiencing incessant or increasingly frequent ventricular arrhythmias. In addition to deactivation, other options for treatment withdrawal are available. Patients and their surrogates may choose not to replace devices as their generators become depleted.^45,⁴⁶

Further, partial or selective deactivation of therapies is an option. Patients or surrogates can determine whether they wish to terminate the ATP or CRT components, or only the shocking function. The complex interaction between determining a patient’s goals of care and the options for CIED management shows that there is no “one size fits all” approach to decision making in patients with advanced illness. Instead, only by clearly examining a patient’s understanding and desired (and undesirable) outcomes can clinicians work with patients and families to ensure appropriate device management at the end of a patient’s life to enhance quality of care while maintaining function and reducing symptom burden.

Discussion of Potential Alternative Treatments

Case 7

A 21-year-old man presented with recurrent syncope caused by polymorphic ventricular tachycardia and received a defibrillator as therapy. Multiple shocks caused by recurrent episodes of torsades de pointes (electrical storm) resulted in his request to have the defibrillator removed. Prolongation of the number of intervals to detect, as well as eventual success of empirical antiarrhythmic therapy, resulted in resolution of the episodes of torsades de pointes and resultant shocks. The patient withdrew his request for defibrillator removal.

As part of the decision-making process for deactivation of some or all CIED therapies in a particular patient, the physician should consider and advise the patient of alternative therapies that might impact the decision. For example, patients with recurrent ventricular arrhythmias resulting in painful ICD shocks may be candidates for reprogramming of ATP therapies, catheter ablation, or pharmacologic treatments and may benefit from referral to a center with expertise in these techniques. Patients with worsening congestive heart failure may be candidates for advanced therapies such as left ventricular assist devices (LVADs) or cardiac transplantation.

Supporting Participants in the Decision-Making Process

The Family

Although the ultimate decision regarding treatments rests with the patient (or legal surrogate), conversations about device deactivation optimally occur with the support of the family. Although the primary individuals providing a patient support are generally the partner or blood relative,⁴⁷ it is important for the health care team to understand the patient’s extended support network, both to help maintain the patient’s overall health and for guidance when the patient can no longer make decisions. In addition to the formal written AD, conversations among members of the health care team, patient, and family in early phases of the patient’s course of disease will help put the entire family “on the same page” in terms of the goals of medical care.⁴⁸ Health care providers play an important role in supporting the patient’s surrogate and facilitating communication and support of additional family members.

Health Care Providers

Key members of the patient’s health care team should be included in deactivation discussions. The patient’s electrophysiologist and cardiologist should be included in deactivation conversations whenever possible, both because they are a member of the care team for these patients, and also to assure that all therapeutic options available to meet the patient’s goals can be evaluated. An interdisciplinary approach is essential to support the patient and family. Nurses, social workers, and clergy often play a key role in helping patients understand the device in terms of the overall context of their health and help them better comprehend aspects of their care related to device management.

Psychiatric Consultation

Case 8

A 69-year-old man with coronary artery disease (CAD), left ventricular ejection fraction (LVEF) of 30%, and New York Heart Association (NYHA) Class I received a prophylactic ICD 2 years earlier. He has never used the ICD. After his wife dies, he comes to clinic and asks that his ICD be turned off.

Case 9

A 20-year-old woman with tetralogy of Fallot underwent surgical repair at age 6 complicated by congestive heart block requiring a permanent pacemaker (PPM). She was married at age 19, but less than 1 year later divorced and returned to live with her mother. She comes to clinic and asks that the pacemaker be turned off.

Routine psychiatric consultation is not needed for patients who are considering device deactivation. It should be reserved for cases where health care providers or families have concerns that a particular psychiatric disorder may be interfering with the patient’s ability to make informed decisions. Examples include major depression, where a “death wish” exists in the patient with untreated depression or thought disorders (e.g., paranoid delusions). For example, in cases 8 and 9, the potential for depression should be evaluated by a psychiatrist. Neuropsychiatric disorders, including delirium and dementia, can also impact decision-making ability. When in doubt, a psychiatric consultation can help the team assess for adequate decision-making ability at a specific point of time.

Palliative Care Specialists

Case 10

A 74-year-old man (JE) was admitted to the cardiac care unit in cardiogenic shock. He was intubated, an intra-aortic balloon pump was emergently inserted, and multiple vasoactive agents were started. JE was weaned from the intra-aortic balloon pump 3 days later and extubated 1 week later. However, JE’s condition progressively worsened, with the onset of sepsis and early signs of renal and liver failure. His level of consciousness vacillated between periods of confusion and periods of lucidity. Several family meetings occurred, and working with the palliative care team, a decision was made to stop aggressive treatments. A fentanyl drip was started at 2 mg/hr, and all JE’s vasoactive agents were stopped and ICD therapies deactivated. Within minutes, JE had chest pain, which diminished after he received further fentanyl. His opiate drip was gradually titrated until his chest pain was relieved. JE died 4 hours later.¹⁷

Palliative care relieves suffering and improves quality of life for patients with advanced illness and their families. Unlike hospice care, palliative care can be provided simultaneously with appropriate life-prolonging therapies.⁴⁹ Most U.S. hospitals have some form of a palliative care program.⁵⁰ Because changing or deactivating device settings can result in gradual worsening of chronic symptoms or onset of new symptoms, it may be helpful to involve palliative care in the care of patients before devices settings are altered, because these concerns can often be eliminated with early symptoms assessment and treatment.

In addition to symptom management, palliative care clinicians are experts at complex conversations surrounding progressive illness. Their involvement in discussions about CIEDs helps to ensure that the patient and family have the opportunity to understand fully the nuances of these decisions and the implications for device management for the patient. Studies show that patients who receive palliative care are more likely to have their treatment wishes followed and have better QOL at the end of life.³⁴ Palliative care also plays a key role in supporting families of patients with advanced disease, who themselves undergo declines in physical and mental health and have an increased risk of death compared with nonfamily controls.⁵¹^–⁵³

Discussions about deactivation may be misconstrued by patients and families as the beginning of abandonment. They must understand that even if they choose to deactivate the device, the clinicians involved in their care will continue to work with them to ensure that their needs (physical and otherwise) are met. Palliative care can assist with safe and seamless transitions from one system of care to another.

Hospice care is provided to patients with a prognosis of 6 months or less to live who have decided to forgo all treatments aimed at curing their underlying terminal illness.⁵⁴ Hospice clinicians should be included in conversations for patients as they near the end of life to ensure continuity in implementing the goals of care regarding a CIED. Currently, most hospices do not have practices in place to ensure that these conversations occur at enrollment. Both specialists and generalists must partner with hospices to facilitate these conversations and ensure the availability of clinicians who can deactivate CIEDs for patients near the end of life.

Further, both hospice and palliative care clinicians can help clarify concerns and misperceptions about the device after the patient has died. For example, some families have erroneous concerns that they may be shocked by touching their deceased relative, or that the device must be explanted after death (which is only true in the case of cremation). Likewise, the clinician can dispel the myth that continued pacemaker function after a patient has died is unnecessarily prolonging life, or even that the impulses from the device are capturing or affecting the heart in some way.

Improving Communication about End-of-Life Care: Importance of Education

To improve communication about device management for patients with advanced disease, educational endeavors need to be instituted for both health care professionals and trainees. Ongoing education for clinicians in practice, including physicians, nurses, social workers, clergy, and device manufacturer representatives, should incorporate teaching about the importance of conversations on device management to improve communication skills and create practice change. Training programs for health care professionals have been shown to improve their knowledge and increase the likelihood they will put new skills into practice.⁵⁵^–⁵⁷ In addition, fellows and other clinicians in training must also learn the importance of these conversations, as well as undergo training specifically aimed at improving skills in communication. Senior health care providers modeling these conversations for learners are key to improving trainee education about these complex discussions.

Logistics of CIED Deactivation

On notification by the patient or a health care provider, the patient’s attending physician then assumes responsibility for addressing the request, counseling the patient, and making a written order in the patient’s medical record. In many cases the attending physician will require consultation with the patient’s cardiologist or cardiac electrophysiologist to determine the specific CIED therapies that are to be deactivated. These specifics should be a part of the written order.

Although the nuances of medical practice require a tailored approach to each patient, HRS recommends that the following series of procedures be consistently applied ⁴:

1 Confirming mental capacity requirements to make the decision to withdraw CIED support.

The responsible clinician should assess whether the patient or surrogate adequately understands the facts of the patient’s medical condition and the likely consequences of withdrawal of therapy, and that the patient is free of coercion by others. Accurately gauging patient understanding in this context requires that the responsible clinician be qualified to discuss in detail the benefits and any potential negative effects of ongoing device therapy. Depending on the clinical context, this may require consultation with a clinical electrophysiologist, if one is not already directly involved in the patient’s care. Patients who have psychological or cognitive problems that may benefit from counseling or pharmacologic therapies should have these addressed before deactivation proceeds.

2 Identifying the legal surrogate if the patient lacks capacity.

3 Ensuring documentation requirements for withdrawing or withholding a CIED.

Deactivation of CIED therapies requires a written order from the attending physician. This should preferably precede deactivation. In emergent situations, a verbal order should be followed by written documentation within 24 hours. The person responsible for ordering device deactivation may be the patient’s primary care physician, cardiologist, cardiac electrophysiologist, a hospitalist, or a palliative care specialist. Any of these specialists may be the most appropriate physician for such an order, although collaboration among the patient’s physicians is ideal. The written documentation in the medical record needs to address the following:

a Confirmation that the patient (or legal surrogate) has requested device deactivation.

b Capacity of the patient to make the decision, or identification of the appropriate surrogate.

c Confirmation that alternative therapies have been discussed, if relevant.

d Confirmation that consequences of deactivation have been discussed.

e Specific device therapies to be deactivated.

f Notification of family if consistent with patient’s wishes.

4 Establishing palliative care interventions and providing patient and family support.

For patients at the end of life, device deactivation should be viewed as one of many potential interventions aimed at preventing discomfort or prolonged suffering. Patients must also be offered the full range of palliative measures to treat symptoms associated with the progression of their underlying illness, especially any new symptoms that may emerge from cessation of device therapy. To allow the patient to experience these symptoms without sedation or analgesia is contrary to humane end-of-life care. Clinical care of patients with arrhythmias does not end with device deactivation, and patients may benefit significantly from pharmacologic measures that minimize symptoms.

In addition, the families of patients may often require considerable emotional support, especially if they have acted as the patient’s decision-making surrogate. Setting expectations for family members regarding the consequences and uncertainties of deactivation is imperative. It may be especially important to have a member of the clergy present for patients with a well-defined faith tradition. Formal consultation with palliative care experts is available in most hospital settings and frequently in long-term care facilities or other nonhospital care settings. This may be particularly appropriate when there is any uncertainty about symptom management before and after device deactivation. It is generally appropriate to discontinue rhythm monitoring when pacing therapy is withdrawn.

5 Knowing how to deactivate the device.

Deactivation should be performed whenever possible by individuals with EP expertise, such as physicians or device clinic nurses or technicians. In situations where this expertise is not available (see later), deactivation should be performed by medical personnel, such as a hospice physician or nurse, with guidance from IEAPs.⁵⁸

Pacing therapy, given the caveats indicated for a pacemaker-dependent patient, may be withdrawn by programming to specific modes (OOO, ODO, or OSO). If such modes are not available for the device in question, the rate can be lowered and the output adjusted to subthreshold levels so as to render the pacemaker nonfunctional. Deactivation of shocking and ATP functions in an ICD may be accomplished by reprogramming of the device or continuous application of a magnet over certain pulse generators. Notably, there may be differences in the response to magnet application among different manufacturers’ devices and individual device programmed features. This further emphasizes the importance of consulting individuals with EP expertise to ensure that the process is as smooth as possible.

Since the most urgent need for deactivation of CIEDs is the situation of repetitive ICD shocks, the recent HRS statement on deactivation suggests that, for patients who are diagnosed with a terminal illness, consideration should be given to providing them with a doughnut magnet and that they be given detailed instructions on its use.⁴ Application of a magnet over ICDs usually will temporarily suspend antitachycardia therapies while not disabling bradycardia pacing functions. However, it should be emphasized that although ICD shocks may be very painful and frightening, they may be lifesaving; therefore, deactivation of the device may not be warranted even in the presence of repetitive shocks, unless the patient has made the decision to forego further device therapies.

Role of Industry-Employed Allied Professional

In many situations, IEAPs may be asked to assist available medical personnel in deactivation when EP expertise is not available. The role of the IEAP is to provide technical assistance to medical personnel,⁵⁸ who will then perform actual deactivation. Available data from a survey of HRS members and IEAPs suggests that IEAPs perform deactivation 50% of the time,² the recent HRS statement on deactivation recommends that the IEAP should always act under direct supervision of medical personnel.⁴ IEAPs experience significant role conflicts when asked to perform clinical functions, in particular device deactivation,⁵⁹ as illustrated by the following quotes from IEAP interviews⁵⁹:

I never physically ever hit the program button. I will set it all up, and then I will have someone hit it … if that makes me sleep at night, maybe that is what it is.

The first time I ever had to do it, I was fine until I walked to my car and thought, ‘Oh my God.’ I just lost it when it was over with. Everyone has got their war story … where they almost couldn’t be professional about it or they almost couldn’t do their job.

Most IEAPs are comfortable deactivating all ICDs, but many are not in the case of pacemakers. Clinicians must provide medical supervision to IEAPs in performance of device deactivation.

Each manufacturer has policies for their personnel that apply to deactivation of CIED therapies, and it is the responsibility of IEAPs to ensure adherence to these policies. If the representative is asked by the patient and the attending physician to deactivate therapies that conflict with the policies of the company, the IEAP has the right to object to programming of the device. In this situation the attending physician assumes responsibility to find another mechanism for device programming, usually by contacting the physician who implanted or who follows the patient’s CIED.

Communication with IEAPs by medical personnel at the scene, as well as physicians with EP expertise, needs to include specific instructions regarding features to deactivate, as well as information about the patient’s overall goals. Indeed, once the IEAP has a better understanding of the purpose of changing a device’s settings, the representative may be able to provide suggestions or clarify misperceptions. IEAPs stress this in interviews: “[Clinicians] should not say, ‘Turn off device,’ but they should say, ‘Disable or turn off—discontinue all tachyarrhythmia therapies. …”⁵⁹

Considerations in Specific Clinical Settings

How the request for deactivation is handled and who will perform deactivation often depends on the setting: (1) acute care hospital with EP expertise, (2) acute care hospital without EP expertise or nonhospital health-care facility, (3) at home. For each of these settings, the initial steps previously described are the same.

Device Deactivation in the Pediatric Patient

A full discussion of end-of-life issues in minors is beyond the scope of this chapter. However, patients age 24 or under account for 1% to 2% of device implants,⁶⁰ and it should be recognized that there will be unique situations where quality survival may be unattainable even for a child, and a “good death”⁶¹ may be the more appropriate goal. Two major questions may arise when one is considering forgoing life-sustaining treatment for a seriously ill juvenile patient: should the young person be informed about the gravity of his illness? And if so, to what extent should that young person participate in the end-of-life decision? Management of CIEDs in children nearing end of life or requesting withdrawal of treatment requires an assessment of the child’s decision-making capacity. Whether or not the child has capacity, communication of decisions should be provided to the child, recognizing developmental level and individual preferences. An ongoing dialogue is required with the juvenile patient, in which his or her concerns are probed and assurance is given that any questions about the illness and its treatment will be answered truthfully. If a child does not have decision-making capacity, a parent or guardian should make decisions in the child’s best interest. (See also Chapter 18.)

Withholding Device Therapy

A second, less frequently-discussed ethical aspect of device therapy lies in the decision to withhold this therapy from a patient who may not benefit or in whom benefit is less clear. The ACC/AHA guidelines for device-based therapy clearly state that “recommendations for consideration of ICD therapy, particularly those for primary prevention, apply only to patients who … have a reasonable expectation of survival with a good functional status for more than one year.”⁴³ However, as recently described by Beauregard,⁶² palliative care experts may be consulted regarding device deactivation in patients with more recent implants. How often this occurs and why are unknown. The question has been raised whether implanting electrophysiologists may ignore severe comorbidities that might limit the benefit of ICDs.⁶² However, in one reported series of patients requesting deactivation, the three patients diagnosed with malignancy before device implant had all been given life expectancy of longer than 1 year by their oncologist.⁶³ Ethics requires that physicians not impose on patients therapies that may be medically futile, as outlined by the AMA Code of Ethics.⁸ However, “medical futility” may be difficult to determine. A “reasonable expectation of survival for more than one year,” a relatively “hard” endpoint, can already be difficult to predict in many cases; “reasonable expectation of survival with good functional status” is even more difficult to define.

To prevent the situation of device implantation in individuals who may shortly be requesting deactivation, it is important first for the physician to weigh carefully the ICD benefit in the context of the patient’s overall medical condition. Also, similar to deactivation discussions, discussions about initial implant should also follow patient-centered, “shared decision-making” models.⁶⁴ Especially in older individuals, discussion of ICD implantation should focus on overall goals of care for a patient’s remaining years.

References

1 Goldstein NE, Lampert R, Bradley E, et al. Management of implantable cardioverter-defibrillators in end-of-life care. Ann Intern Med. 2004;141:835-838.

2 Mueller PS, Jenkins SM, Bramstedt KA, Hayes DL. Deactivating implanted cardiac devices in terminally ill patients: practices and attitudes. Pacing Clin Electrophysiol. 2008;31:560-568.

3 Goldstein N, Bradley E, Zeidman J, et al. Barriers to conversations about deactivation of implantable defibrillators in seriously ill patients: results of a nationwide survey comparing cardiology specialists to primary care physicians. J Am Coll Cardiol. 2009;54:371-373.

4 Lampert R, Hayes DL, Annas GJ, et al. Heart Rhythm Society Expert Consensus Statement on the management of cardiovascular implantable electronic devices (CIEDs) in patients nearing end of life or requesting withdrawal of therapy. Heart Rhythm. 2010;7:1008-1026.

5 Beauchamp TL. Principles of biomedical ethics, ed 6. New York: Oxford University Press; 2009.

6 Annas GJ. The rights of patients: the authoritative ACLU guide to the rights of patients, ed 3. New York: New York University Press; 2004.

7 Snyder L, Leffler C. Ethics manual, fifth edition. Ann Intern Med. 2005;142:560-582.

8 American Medical Association Council on Ethical and Judicial Affairs. AMA 2008–2009. Code of Medical Ethics: current opinions and annotations. Chicago: AMA Press; 2010.

9 Pellegrino ED. Decisions to withdraw life-sustaining treatment: a moral algorithm. JAMA. 2000;283:1065-1067.

10 Rhymes JA, McCullough LB, Luchi RJ, et al. Withdrawing very low-burden interventions in chronically ill patients. JAMA. 2000;283:1061-1063.