Implantable cardioverter-defibrillator complications

Studies in this population have shown that painful inappropriate shocks lead to anxiety, fear, and anticipation of subsequent shocks. Children and patients with CHD have a higher risk of receiving inappropriate shocks, ranging from 19 to as high as 46% in certain higher-risk groups,Reference Kamp, Von Bergen and Henrikson ^{7 –} Reference Von Bergen, Atkins and Dick ⁹ compared with only 12% in adult patients with implantable cardioverter-defibrillators.Reference Alter, Waldhans, Plachta, Moosdorf and Grimm ¹⁰

Lead fracture and failure in children and patients with CHD typically require additional surgical intervention and, in some cases, lead extraction. These procedures add risk, especially in those who are small or with distorted anatomy, thereby contributing to morbidity and risk of mortality. Lead failure occurs in up to 16% of paediatric and CHD implantable cardioverter-defibrillator patients.Reference De Witt, Triedman and Cecchin ^{11 ,} Reference Atallah, Erickson and Cecchin ¹² The risk of lead fracture in paediatric and CHD patients was evaluated in a large multicentre study of this population.Reference Atallah, Erickson and Cecchin ¹² Recalled leads have resulted in an increase in complications in this population in the past 10 years. These recalled leads contributed to a much higher risk of lead fracture, 9.1% per year compared with 2.3% per year for non-recalled leads. Even if one excludes recalled leads, the risk of lead fracture is about two times higher in children and patients with CHD compared with adults, in whom the risk is 0.6% per year. In a recent study of children with primary prevention indications for implantable cardioverter-defibrillator implantation, overall adverse events occurred in 36% of patients. In addition, this study showed that the risk of adverse events was higher than the incidence of appropriate shocks.Reference De Witt, Triedman and Cecchin ¹¹ Lead revisions and other complications may require surgical intervention and hospitalisation, which imposes further disruption in the lives of implantable cardioverter-defibrillator patients, potentially contributing to psychosocial dysfunction and poor acceptance of the device.

Activity restrictions

A specific and significant contributor to optimal psychosocial functioning for many patients is the ability to participate in athletic activities. Many paediatric and CHD patients may be restricted from sports related to either the underlying diagnosis or the presence of an implantable cardioverter-defibrillator, thereby negatively impacting these patients.Reference Rahman, Macciocca, Sahhar, Kamberi, Connell and Duncan ¹³ These restrictions were recently addressed in the 2015 American College of Cardiology/American Heart Association-sponsored Eligibility and Disqualification Recommendations for Competitive Athletes with Cardiovascular Abnormalities.Reference Zipes, Link and Ackerman ¹⁴ Patients with an implantable cardioverter-defibrillator are able to participate in Class IA sports such as golf, bowling, cricket, billiards, riflery, and curling if they are free of episodes of ventricular tachycardia or ventricular fibrillation. Participation in higher than Class IA sports may be considered if the athlete is free of symptoms of ventricular tachycardia or ventricular fibrillation requiring implantable cardioverter-defibrillator therapy for 3 months. These new guidelines support the concept of patient and family autonomy, recommending that the decision to participate should be based on discussion with the family and patient about the potential risks of shock and injury to the implantable cardioverter-defibrillator system. Although the guidelines support participation, the desire for an athlete to continue with sports should not be the sole reason for implantation of an implantable cardioverter-defibrillator. Although guidelines are more liberalised, patients are often still restricted from at least some activities that they enjoy, contributing to psychosocial dysfunction.

Patient and family education

Directly related to patient autonomy is the importance of empowering the patient with knowledge about the device, the disease, and the risks of both. Education should start before finalising the decision to implant, in order to support understanding and acceptance by the patient and family. It should include discussion of the perceived risk of cardiac arrest and the benefit of the implantable cardioverter-defibrillator. Potential complications, including both short and long term, should be reviewed as well as the anticipated school, work, or activity restrictions related to the implantable cardioverter-defibrillator.

Following the decision to implant, there should be ongoing education including implant technique and the type of implantable cardioverter-defibrillator. Keeping the patient informed of such details helps them feel involved in the process and promotes acceptance. Postoperative care including use of antibiotics, postoperative pain control, and temporary mobility restrictions such as avoiding overhead reaching should also be addressed. The follow-up schedule and education on remote monitoring should be discussed. Patient and families appreciate education from an early stage as they can anticipate and plan for issues and follow-up. Ongoing education including review of restrictions and compliance with remote monitoring and follow-up should be reviewed at each clinic visit. This is especially important during the transition to young adulthood as the patient becomes more autonomous and may require re-education.

Resources for education should be communicated to families and updated as needed. Sources of education include industry websites for technical answers on a patient’s specific device. Websites such as asktheicd.com, the website of the Pediatric and Congenital Electrophysiology Society (pediatricepsociety.org), and that of the Heart Rhythm Society (hrsonline.org) can provide an overview of implantable cardioverter-defibrillators and their use.

The goal of minimising shocks should be a priority. Whenever appropriate, programming should include lengthening of the detection time and increasing the detection rate in order to help prevent inappropriate shocks. Antitachycardia pacing therapies, supraventricular tachycardia discriminators, and multiple detection and treatment zones have also been shown to reduce shocks.Reference Saeed, Hanna and Robotis ^{15 –} Reference Mastenbroek, Pedersen, van der Tweel, Doevendans and Meine ¹⁸ Anti-arrhythmic medication compliance should be encouraged to help reduce appropriate and inappropriate shocks. Finally, acute treatment of inappropriate shocks with magnet application should be discussed. In some instances, providing the family a magnet accompanied by education about its use may be helpful to prevent multiple inappropriate shocks.

Patient and family support

Although psychosocial dysfunction varies from patient to patient, all patients having an implantable cardioverter-defibrillator need some level of support. Some patients may have no or mild dysfunction, and some patients are significantly affected. Some patients seek support and others prefer to find support within their family; however, the need for psychosocial support should be assessed before and after implantation of the implantable cardioverter-defibrillator and with each clinic visit. When necessary, support should be offered and tailored to the individual. Ongoing resources for support include social media and other local chat boards. For those who require or wish to have more face-to-face support, support groups and support events can be helpful. They are low-cost groups and are relatively simple to arrange. Support groups should be led by personnel familiar with this type of intervention and with knowledge of device and medical issues. Support groups are ongoing, whereas support events occur less often.

The Young ICD Connection is a collaborative support event hosted by the University of Michigan Congenital Heart Center and the University of Michigan Cardiovascular Center. The event is now in its 20th year, and, although it targets younger patients with implantable cardioverter-defibrillators, patients of all ages attend and are welcome. The majority of attendees have CHD, hypertrophic cardiomyopathy, or a primary arrhythmia syndrome. The event occurs annually on a weekend and is free for families; financial support is obtained through grants and philanthropy. Scholarships are available for patients with financial hardship who must travel long distances. The programme includes support groups for patients with implantable cardioverter-defibrillators, parents, spouses, and friends of patients with implantable cardioverter-defibrillators. Trained social workers and other volunteers lead these support groups. Children and adolescents, including siblings and friends of patients with implantable cardioverter-defibrillators, participate in various team-building and other fun activities. Formal education is provided by selected keynote speakers who address various important issues such as transition of care, lead recall concerns, device education, and other topics. A formal question-and-answer session is conducted by a panel of experts for adult patients and family members, whereas children participate in a focussed activity. During this time, teenagers are able to connect through a structured support session led by members of the clinical team and trained social workers. Informal education is offered throughout the day by volunteer clinical staff. Lunch time and other socialising time allow for informal support from others who share similar experiences. In addition, patient volunteers share stories of their experience with an implantable cardioverter-defibrillator in a group setting. Patients express their appreciation for a structured way to offer and receive support from others who have similar experiences. Adolescent attendees often develop lifelong friendships and have continued support outside of scheduled events. There are similar programmes across the country and can be utilised by patients who express a need for this kind of support.

Another important psychosocial support resource specifically for the younger population is specialty camps. These camps are structured similarly to other day and residential camps offering many activities such as water sports and arts and crafts. These camps are designed to provide a traditional camp experience with the added protection of a full-time, specialty-trained medical staff. Patients participate as they are able, and otherwise can participate in alternate activities if they are restricted because of their disease. Children who attend describe a feeling of normalcy and appreciate the understanding and the friendship from others with a similar background. These types of camps are located throughout the country and are relatively easy to access. In many instances, the cost is free or minimal.

Mentors or peer support can be helpful for young patients as more of an ongoing support system. Peer support is provided by patient volunteers who have an implantable cardioverter-defibrillator and wish to mentor another patient with a similar situation. The relationship becomes reciprocal and helpful for both parties. Age and sex matching these patients is necessary for bonding. As with the bonding in support groups or support events, lifelong relationships can form providing support outside of events and support groups. The healthcare provider who facilitates this relationship must screen individuals to avoid a negative relationship. Recruiting optimistic and generally positive patients is ideal for this type of support.

Cardiac rehabilitation can be helpful for active patients who would like to exercise but who require a controlled environment. Cardiac rehabilitation traditionally focusses on older adults, making it difficult for adolescents and children to find a location that supports a younger client. Specialty children’s healthcare facilities may provide this, but insurance coverage should be assessed before referral.

For patients who require more formal treatment from mental healthcare providers and who may require medical intervention, identifying a psychiatric provider who is local and who has knowledge of device and medical issues is ideal. In some centres, this psychiatric care provider has a close relationship with the cardiology clinical staff and may even be part of the department. In other centres where there are no such specific providers, the clinical staff is instrumental in helping arrange this type of care for young patients close to their home.

For patients who have significant psychosocial dysfunction, psychosocial therapies such as cognitive behaviour therapy has shown some promise in promoting adaptation and acceptance of the device within the first year after implant in adults.Reference Irvine, Firestone and Ong ¹⁹ Cognitive behaviour therapy may be difficult to access without a dedicated psychiatric team and may be difficult to translate into clinical practice. Mindfulness Based Stress Reduction is a structured psychosocial educational programme that includes yoga, meditation, and a support group and has been successful in adults with implantable cardioverter-defibrillators. In a recently published pilot study of 10 children (15±3 years; 60% male), patients participated in a weekly session (each lasting 1–1.5 hours) for 6 weeks.Reference Freedenberg, Thomas and Friedman ²⁰ The programme was led by a clinician trained in the Mindfulness Based Stress Reduction curriculum. The intervention included sessions to train the patient on coping mechanisms such as deep breathing, guided imagery, and yoga. Group discussion was also an integral part of the intervention. Study questionnaires were completed before intervention and on the last day of intervention. Following the 6-week Mindfulness Based Stress Reduction programme, patients reported a decrease in the frequency of anxiety and overall decrease in anxiety.