Abstract
The number of annual implantable cardioverter defibrillator (ICD) implants has substantially increased over the last 5 years and is expected to grow rapidly. Implantable cardioverter defibrillators have a proven mortality benefit by terminating the life-threatening arrhythmias, even near end of life. In patients with moderate/severe symptomatic heart failure, enough clinical literature representing mortality benefits has been published, but limited numbers of studies have reviewed the dwindling risk–benefit profile near end of life, studying quality of life (QoL)/psychosocial impact. Criteria outlining either continued use or deactivation policy/procedures near end of life have not been clearly defined and/or largely implemented, which in turn requires more focused research using multifactorial approach to determine improved patient-centered outcomes.
Keywords
Background Review
Since the inception of external defibrillation in 1960s and implantable cardioverter defibrillators (ICDs) in early 1980s, a paradigm shift has been observed in the management approach for sudden cardiac death (SCD). Each year, 300 000 people die from SCD or 807 people die of SCD daily (American Heart Association [AHA] 2007). 1 This number accounts for more deaths when compared with combined mortality from stroke, lung cancer, or breast cancer. Implantable cardioverter defebrillators have been designed to detect and treat life-threatening arrhythmias. Implantable cardioverter defebrillators are recommended in patients with Grade II/III New York Heart Association (NYHA) heart failure.
Several published studies since 2002 on the review of ICDs associated with patient-centered outcomes (quality of life [QoL] anxiety, depression, distress) were potentially unable to correlate ICD and poor QoL. 2,3
The number of annual ICD implants has substantially increased over the last 5 years and is expected to grow rapidly. Around 3 million people in North America are now eligible for ICDs and 400 000 additional meet these criteria every year. Implantable cardioverter defebrillators have a proven mortality benefit by terminating the life-threatening arrhythmias. Various studies including AVID (antiarrhythmics vs implantable defibrillators) trial showed that 1-year survival rate was 89.3% for patients who received ICD implants versus 82.3% for patients treated with drug therapy. 4,5
Sudden cardiac death in heart failure (SCD-HeFT) trial demonstrated the all-cause mortality benefit from ICD in NYHA classes II and III patients with ejection fraction (EF) <35%. Amiodarone had no effect on all-cause mortality; in fact, there was an increase in noncardiac mortality in NYHA class III as eluded by various studies. 4,5 Apart from this, in some published studies a relative risk reduction of 20% to 30% mortality has been seen in patients with no previous history of tachyarrhythmias (primary prophylaxis; Table 1).
Summary of the Trials for ICD and Associated Mortality Benefits
Abbreviations: ICD, implantable cardioverter defibrillator; NYHA, New York Heart Association; EF, ejection fraction; VF, ventricular fibrillation; VT, ventricular tachycardia; PVC, premature ventricular contractions; RRR, relative risk reduction; NSVT, non sustained ventricular tachycardia.
Data adapted from Salukhe et al. 6
Through this article, we intend to review the implant procedures and associated functioning of ICDs, guidelines formulated by the joint task force (AHA, American College of Cardiology [ACC], and Heart Rhythm Society [HRS]) for implanting ICDs, relationship of hospice and ICD implantation as predictors of QoL in these patients, when/how to deactivate/turn off the ICD, how far have these policies been implemented, and how to improve the standard of care to achieve a good QoL.
Implantable Cardioverter Defibrillator
An ICD is a mix of a pulse generator and a pacing lead. Implantable cardioverter defibrillator generator is larger when compared to a conventional pacemaker since it carries a larger battery, large capacitor, and a more complex microprocessor. Pacing lead consists of a pacing tip electrode and 1 or 2 integrated defibrillator or shock coils that are positioned in the right ventricle (RV) and superior vena cava (SVC). An ICD is implanted via transvenous (subclavian, cephalic, or axillary veins) access from an incision below clavicle and medial to deltopectoral groove. The generator rests under the skin in the pectoral region usually under the clavicle on either side of the chest. This includes an electrode wire that is advanced through a vein, axillary, subclavian, or cephalic, to the right side of the heart. Subcutaneously placed ICDs are being evaluated in various trials and are not yet approved. 1,3
Heart rate and rhythm is constantly monitored through ICDs for any variations and preset programming allows an electrical shock to be delivered to curb any abnormal rhythms. The device is capable of terminating the arrhythmias either with antitachycardia (overdrive) pacing (ATP) or with cardioversion and defibrillation. Antitachycardia pacing refers to the delivery of short bursts of rapid ventricular pacing to terminate ventricular tachycardia (VT). It is comparable in efficacy to low-energy cardioversion and is painless. 7 Cardioversion is shock that is delivered at the peak of the R wave. Benefit of cardioversion are observed in terminating organized rhythm-like VT but not ventricular fibrillation (VF) which requires the use of unsynchronized shock called defibrillator. Apart from terminating arrhythmias, ICD assist withpacing as well. Implantable cardioverter defibrillator have been studied and observed to terminate at least 97% of the ventricular arrhythmias. All ICD generators store intracardiac electrograms for recording abnormal rhythms and delivering therapies which are available to download either in person or remotely through transtelephonic monitoring (Figure 1).

A fully implanted implantable cardioverter defibrillator (ICD) in a patient. 8
Electromagnetic Interference
Electromagnetic interference is defined as the interference between the device and any potential source of electromagnetic field, ionizing radiation, or acoustic radiation. Patients may come in contact with electromagnetic field from sources like cellular phones, welding equipment, improperly grounded appliances, slot machines, electric motors, electric razors, high-voltage power lines, surveillance devices, magnetic resonance imaging, electrosurgery, and diathermy involuntarily and may be subjected to the adversity though the incidence of this interaction is quite unlikely (<1%) as reported by various studies. 9 It has been advised that there are minimal chances of interaction to occur between the cellular phones and implanted ICD devices if the distance between the phone and the device is at least 6 inches. It has also been recommended not to use cellular telephones during ICD programming. Patients can even walk through metal detectors but handheld detectors should be kept away from these devices. Antitheft systems are another challenge but it is advisable for these patients if they walk at normal pace and do not linger in proximity or lean against the antitheft system. Patients should at least keep 2 feet distance from external electrical equipment and make sure it is properly grounded to avoid any involuntary adversity due to the interactions.
Acoustic radiation in the form of lithotripsy is considered to be unrelated to any interactions if the device is turned off during the treatment and appropriate cardiac monitoring is performed. 10,11 Magnetic resonance imaging (MRI) is still a contraindication for ICD patients. Patients having noncardiac surgery where electrocautery is used may have device malfunction and device should be turned off immediately before surgery, reassessed, and turned back on immediately after the operation. An external defibrillator should be made available while the device is off. 12 Limited data are available on the management of ICD patients undergoing radiation though the modern devices are more sensitive to radiation due to the incorporation of complementary metal oxide semiconductor (CMOS). Data are available in the form of case studies only. Hudson et al reviewed data on interaction of both pacemakers and ICDs, making it clear that device malfunction is rare and death associated with it is even more uncommon. There are no data to prove that the current guidelines are adequate to prevent device malfunction. It is important to consider accumulated dose of radiation, backscatter, dose rate, and fractionation. Each oncology and radiation department should have a policy to manage patients with ICDs who are undergoing radiation based upon national and international standard. 13 Precautions should be taken while having dental treatments using ultrasonic scaler, ultrasonic cleaning system, and battery-operated composite curing light as they may have deleterious effects on the device. 14 Laser eye treatments have been reported safe though the data regarding this are limited. 15
Device Therapy Indications
Based upon 2008 guidelines from ACC/AHA/HRS for device-based therapy, ICDs are indicated in patients who have survived cardiac arrest due to VF/VT but per the indications, this may not include the patients who developed VF within 48 hours of an episode of acute MI. Patients with structural heart disease who may develop spontaneously sustained VT qualify to be a potential patient for ICD. Patients who develop syncope with no determined underlying etiology in electrophysiology (EP) studies with inducible VT/VF should get ICD. Ejection fraction <35%, NYHA class II or III, and patients who are not in a window period of 40 days from myocardial infarction qualify for an ICD. The underlying cause for low EF could be ischemia or nonischemic dilated cardiomyopathy. Patients who belong to NYHA class II or III and have a low EF of <35% must have expected survival of more than one year to get an ICD. In cases where the intraventricular conduction delay of >120 ms and a low EF of <35% has been observed, these patients may need ICD with biventricular pacing function.
Patients who have hypertrophic cardiomyopathy with one or more major risk factors for SCD qualify as a potential patient to receive an ICD (class IIa per the ACC/AHA/HCS guidelines). Major risk factors as identified for SCD are prior cardiac arrest, sustained VT, and sudden death of a first-degree relative younger than 40 years old. Brugada syndrome, characterized by a peculiar pattern on the electrocardiograph (ECG) consisting of a pseudo-right bundle branch block (RBBB) and persistent ST segment elevation in leads V1, V2, and V3, is a significant risk factor for cardiac arrest and hence patients should get ICD if they develop syncope or VT (class IIa). Patients with long QT syndrome who develop syncope or VT while on beta blockers need ICD (class IIa). Class IIa indications may include patients with arrhythmogenic RV cardiomyopathy (with one or more risk factors for SCD), sarcoidosis, Chagas disease, giant cell myocarditis, catecholaminergic polymorphic VT (who develop VT or syncope while on beta blockers), sustained VT with normal EF, and patients who are awaiting heart transplantation (Adapted from AHA/ACC/HRS 2008 Guidelines). 1,4,5,16 –31
Complications
Like any other operative procedure, placement of an ICD could lead to bleeding, dislodgements, and infections, pneumothorax, and cardiac perforation. Complications related to lead include lead infection, lead malfunction, and lead fracture. Pacemaker infection has been reported, leading to removal and getting a new one on the other side. 32,33 Twiddler syndrome is twisting of the device in its pocket, leading to dislodgement and malfunction. Patients may get inappropriate shocks due to supraventricular tachyarrhythmias and nonsustained VT. 34 A case of rhabdomyolysis from repeated shocks has also been reported. 35 Various cases of staphylococcal infection at earlier as well as at later stages of implantation have been reported associated with prolonged hospital stays. Based on the results reported by Krahn et al, 1.2% of the patients undergoing ICD replacement reported pulmonary edema, 1.3% patients reported electrical storm and 1% reported pocket hematoma and incisional infection (Table 2). 36
ACC/AHA/HRS Indications for Placement of Implantable Cardioverter Defibrillators a
Abbreviations: ACC = American College of Cardiology; AHA = American Heart Association; EF = ejection fraction; HRS = Heart Rhythm Society; MI = myocardial infarction; NYHA = New York Heart Association; VF = ventricular fibrillation; VT = ventricular tachycardia.
a Data adapted from Epstein et al. 5
b Class of recommendations: I = benefit > > > risk, procedure should be performed; IIa = benefit > > risk, reasonable to perform procedure; IIb = benefit ≥ risk, procedure may be considered; III = risk ≥ benefit, procedure should not be performed.
c Level of evidence (estimation of certainty [precision] of treatment effect): A = multiple populations evaluated, multiple randomized trials or meta-analyses; B = limited populations evaluated, single randomized trial or nonrandomized studies; C = very limited populations evaluated, expert opinion, case studies, or standard of care.
Implantable Cardioverter Defibrillators and Hospice
Hospice has played a significant role in management of patients with advanced disease or patients near end of life. Hospice actively admit patients near end of life. These patients are highly susceptible to adverse life events and an increased frequency of unwanted shocks due to their general medical condition. Based on the study conducted by Goldstein et al in 2009, it was confirmed that 97% of the hospices accept patients with ICD. In 58% of the hospices, atleast 1 person got shocked in year 2009, and 40% of 58% these institutions reported that at least 1 patient got shocked multiple times during the year 2009. Implementation of the deactivation policies inclusive of an ethical rationale, process of informed consent, and process for deactivation has been observed to a very limited extent though the intended benefit for these patients in terms of the QoL is thoughtfully higher. There could be a possible association between the hospice having deactivation policy and more patients choosing deactivation near end of life and getting lesser shocks. In lieu of this reported data, it is certain that we may improve the patient care near end of life by addressing deactivation policies across the hospices nationwide. 37,38 Unfortunately, criteria defining continued use or deactivation of these devices in patients near the end of life are yet to be seen in relevant published literature reviews (Table 3). 39
Survey Data Collected by Morrison et al on Experiences of Hospices and Palliative Care Clinicians on Managing ICDs Near End of Life a
Abbreviation: ICD, implantable cardioverter defibrillator.
a Data adapted from Morrison et al. 40
Quality of life and Psychosocial Impact
Quality of life index has been closely associated with ICDs implanted in patient population near end of life, and several studies have determined poor QoL with associated multiple shocks but causality of this relationship is still considered to be a gray area. Patients have elevated levels of anxiety and depression resulting from the fear of ICD discharge, device failure, decrease in physical activity, and negative lifestyle changes such as the inability to drive. More the number of ICD discharges, more is the likelihood of patients getting anxiety and depression and reduced QoL. 41 Preimplant psychological variables like anxiety, depression, low optimism, and lack of social support account for as much as age and EF for variance in QoL outcomes. Psychological interventions in all these cases may be beneficial. 42 Various health care providers and published studies have reported that 38% patients reported better QoL, 47% patients reported the same QoL and 15% of patients reported worse QoL post ICD implantation. Most common concerns were shocks, depression, and driving restrictions. Our health care providers are comfortable dealing with the medical issues but not emotional well-being. 42
Several studies comparing the benefits of ICD versus medical therapy have not been successful in outlining significant benefits of medical therapy over ICDs. Implantable cardioverter defibrillator use in elderly population has been associated with reduction in all-cause mortality. 43 Improvements in functional status and overall QoL have been reported by various published randomized clinical studies, but most of the studies had limitations pertaining to shorter follow-up. 44 –47
Dougherty and colleagues determined that fear and ICD concern comprises the majority percentage of the associated anxiety which was significantly reduced in the intervention group when compared to the usual care group. 48
Inspite of the extensive published literature and ongoing studies on the use of ICDs and associated benefits in patients near end of life; there are still some unknowns yet to be determined by studies that are not limited by either power or follow-up period in terms of impact of the interventions implemented and varied levels of anxiety.
Deactivation of ICD at End of Life/Ethical and legal parameters
It is both legal and ethical for the patient to request deactivation of an ICD whenever goals of care have changed and patient does not wish to be resuscitated. Deactivation of an ICD with the informed consent of the patient or legal surrogate is not physician-assisted suicide or euthanasia.
Implantable cardioverter defibrillators have a significant contribution in improving the QoL as well as reducing mortality but if the patient has terminal illness like metastatic cancer that is not reversible, patient and family should be involved in making decisions about end of life and withdrawing ICDs. In one survey, most medical and legal professionals and patients voted for device deactivation in the phase of terminal illness and end of life. 49,50 Clinicians should initiate end-of-life discussions when they are in good health and document their wishes in the form of advance directives. 50,51
When a patient’s request for withdrawal of a life-sustaining treatment that a patient perceives as unwanted (ICDs), physician’s intent is to discontinue the unwanted treatment and allow the patient to die naturally of the underlying disease—not to terminate the patient’s life. 51,52 Informed consent discussion with the patient and family should include the facts that (1) turning off means the device will no longer shock in the phase of life-threatening arrhythmia; (2) turning off the device will not cause death; And (3) death is not going to be more painful with the device turned off. 40 A survey of hospice and palliative care providers also supported the deactivation of an ICD with informed consent from the patient or the legal surrogates. 52,53 Patient autonomy in decision making should be the first and foremost consideration and the pain from an ICD shock can be contrary to a primary goal of maintaining the patient's comfort. Internists are less comfortable discussing deactivation of an ICD as compared to other life-prolonging therapies as they do not have much experience dealing with it. 52,54 Every time an ICD is placed, there should be a detailed discussion with the patient and the family about advance directives and turning off the device at end of life. In one study only one third of terminally ill patients were able to decide to turn off the device, and they had fewer shocks in their last days. 55
Reprogramming or Deactivation of an ICD
Reprogramming or deactivation should be performed as ordered by the attending physician and considering the clinical scenario assuming that either this procedure to be performed by or under physician’s guidance in the hospice or clinic setting or performed remotely. Simply placing a doughnut magnet over the ICD will allow temporary suspension of the antitachycardia therapy but will not affect bradycardia pacing therapy. Deactivation of an ICD can be performed in a clinic, home, or a hospice setting. If ICD cannot sense the rhythm, it will not shock but magnet works till it is physically present over the device. Permanent reprogramming of the device needs it to be done by electrophysiologist or someone with special training in the deactivation of ICDs as well as medical device representatives or field clinical engineers who are thoroughly trained with the functioning of these devices. 52
In patients who presented near end of life as the cause of imminent death and requested deactivation, all antitachycardia therapies, that is shocks and ATP should be deactivated, whereas in patients who are in a stable cardiac condition or presented with slow VT (100-160 bpm), VT may not lead to death but to severe or aggravated symptoms when deactivated. In such patients, the deactivation of shock therapy alone may be preferred. 9
Another approach is not to replace these devices when they are due their elective replacement, and surgical removal of these devices is not recommended as well since it is a painful process associated with higher probability of complications and adverse outcomes in these patients
Debating Good QoL and Managing Medical and Psychosocial Issues
Sears et al did a great deal of work on how to manage patient critical events and achieve desirable QoL. 53 Patient education is so vital to get good results and achieve good QoL. It includes providing them with information about how the device works, what to do if shocked, regular follow-up with the cardiologist, and limitations on part of the patient not to get engaged in certain things which can interfere with the device, like EMI. Reassure patients by giving an example that majority of patients tolerate ICD very well and that they can return to work. Shock is definitely the critical patient event, and it negatively affects the QoL. Modern devices are capable to reduce the frequency of inappropriate shocks with inbuilt mechanisms to distinguish various kinds of mild arrhythmias like super-ventricular tachycardia (SVT) which may be mistaken for VT and patient is shocked. Devices are equipped with various discrimination algorithms and ATP algorithms to prevent inappropriate shocks. Recall events are another blow to the faith in device. Device malfunction is possible and patients should be aware of it. Consider referral to mental health provider in case patients are facing challenges that they cannot handle themselves. Positive communication is really important to enhance confidence and patient compliance. Discuss advance directives with the patient and the surrogates, respect patient autonomy, and patient’s rights to request device deactivation. To accomplish these goals, we need to work as a team including cardiologist/electro physiologist, nursing educator, mental health providers, and regular follow-ups with the electrophysiology clinics and primary care physicians.
Discussion
In patients with stable/moderate symptomatic heart failure, single-lead ICD therapy enhanced survival and was associated with improved health-related QoL. Despite proven benefits in preventing sudden cardiac death, much less ground has been established in preventing harm observed in clinical practice and research in patients nearing end of life. Patients should be assessed for psychological distress and QoL using serial assessments. Patients develop severe anxiety after ICD implantation which has been observed to be associated with ICD shock or progression of their heart disease and hence serial assessments are important.
In lieu of patient-centered outcomes and evidence from small-scale intervention trials, there is a need for psychological intervention in particular with a view to reducing anxiety and concerns about the ICD implantation/deactivation near end of life which may overlook the associated benefits in reducing mortality.
As reported by a study conducted in Europe by Ladwig et al, 56 no clinical institution (96% in Europe) offers routine counseling of ICD patients nearing end of life as well as only 25% of cases, physicians initiate a discussion with the ICD patient. Only about 50% of the patients have significant understanding of the legal aspects of ICD deactivation. This may eventually affect the relationship of a provider and a patient since most of the patients nearing end of life are not able to address this issue or if they intend to, they are reluctant to address this topic and instead rely on their physician’s decision. Due to methodological limitations in ongoing and published studies, more focused research using standardized methodology of multifactorial approach is needed to determine how effectively clinical and psychosocial interventions can relieve emotional distress and improve patient-centered outcomes. Lack of published literature or guidelines for ICD implementation or deactivation for patients near end of life and minimal implementation of existing deactivation policies in all hospice palliative care institutions demand future studies to be large scale power randomized clinical trials to deliver significant results. These intervention trials are necessary in order to provide the most optimal care for the increasing number of patients who receive an ICD near end of life now and in the future.
Footnotes
Declaration of Conflicting Interests
The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding
The author(s) received no financial support for the research, authorship, and/or publication of this article.
