Elsevier

Progress in Cardiovascular Diseases

Volume 51, Issue 3, November–December 2008, Pages 237-263
Progress in Cardiovascular Diseases

Sudden Cardiac Death Symposium
Risks and Challenges of Implantable Cardioverter-Defibrillators in Young Adults

https://doi.org/10.1016/j.pcad.2008.10.001Get rights and content

The clinical use of the implantable cardioverter-defibrillator (ICD) is well established to prevent sudden death in patients with left ventricular dysfunction due to coronary artery disease and dilated cardiomyopathy, and its use has saved thousands of lives. More recently, its use has been extended to other patients at risk for sudden cardiac arrest due to ventricular fibrillation: patients with structural heart diseases such as hypertrophic cardiomyopathy and arrhythmogenic right ventricular dysplasia and patients with normal cardiac anatomy and function except for electrophysiologic abnormalities that predispose to cardiac arrest: Brugada syndrome and long QT syndrome. A distinguishing feature of these patients may be the young age when they present for either primary or secondary prevention. This contrasts with the “first wave” of adult ICD implantations that were usually performed in elderly or middle aged patients. An important consideration in favor of ICD implantation in young patients with sudden death risk is the long and cumulative period of their risk. Similarly, after implantation, these patients will experience the long-term risks of ICD implantation. This review focuses on the unique challenges presented by device implantation in young patients 16 to 45 years of age who may have 4 to 7 decades of life with their devices. Although devices may prolong life, they come with problems that will pose unique challenges for both patients and their physicians. Moreover, because of the long durations, these problems may accelerate as patients age.

Section snippets

Implantation Risk

Unlike risks enumerated below, implantation risk may not be increased in the younger ICD patients with hypertrophic cardiomyopathy, long QT syndrome (LQTS), Brugada syndrome, or other similar conditions since they may have greater cardiopulmonary and renal reserve to survive a rare serious complication. However, because of the potential need for reimplantation of leads after infection or fracture and, routinely, generators, the cumulative risk of implant-related complications will be greater in

Infection

It is estimated that at least 12 000 cardiac device infections occur annually. They require prolonged hospitalization and expensive hardware replacements with per-case treatment costs of up to US $80 000 when an ICD is explanted and a new ICD system inserted.62, 63, 64 The diagnosis and management of cardiac device infection has been reviewed.62, 65, 66

Device infections can be classified in terms of timing and infection location. First, infection may occur early (<30 days after implant) or late

Inappropriate Shocks

Inappropriate shocks are defined as shocks delivered by the ICD for a non-VT/VF rhythm. Etiologies of inappropriate shocks include AF, sinus tachycardia, or other supraventricular tachycardia, oversensing of intracardiac signal, oversensing of diaphragmatic or other myopotentials, and lead failure (fracture or insulation defect) (see Table 3 and Fig 3). The most common type of oversensing is sensing of T waves in addition to the R wave (T-wave oversensing), but less common instances include

Imperfection

Innovation and improvement of both lead and generator systems have marked the 30 years of the ICD era. In the course of improvement though, the search for smaller and more capable devices has infrequently been marred by failures of both leads, generators, and the connections between them.

Lead Failure

Defibrillator leads have been termed their weakest link.165, 166, 167 The first manifestation of lead failure sometimes is the dramatic occurrence of inappropriate shock or a train of shocks. This occurs when a fracture or insulation break causes oversensing of microvolt potentials that are sensed and confused with VF. Catastrophic failure to deliver needed shock or pacing has occurred. Or, patients may present with abnormal electrical parameters detected at routine device check. Wireless

Generator Failure

Maisel175 reported on a meta-analysis of 3 reported series of prospective registries of ICD generator reliability spanning 1988 to 2004. Only malfunctions due to integral component failure, hardware, or firmware were tabulated. During the period 1988 to 1998, there was a progressive reduction in ICD malfunction rates from 50 malfunctions per 1000 patient-years to less than 5 per 1000 patient-years. However, subsequently, there was a spike in failure rates peaking in 2001 at 26 per 1000

Tricuspid Insufficiency

Transvenous pacing wires and ICD leads are positioned across the tricuspid valve most frequently into the right ventricular apex. Lead insertion has been associated with worsened tricuspid insufficiency (TR).180, 181, 182 In an observational study of 248 patients who had baseline mild or no TR, 4% developed severe tricuspid regurgitation 3 months after lead placement.180 ICD leads were more likely to induce TR than pacer leads, as they are thicker and stiffer.180 There was no correlation

Insurance: Risk of Never Using the Device

A potential risk of ICD implantation is long survival, and never using the device. This risk is inversely related to the severity of the underlying myocardial disease and its likelihood for heart failure death.

In heart failure patients ICD implantation decreases sudden death mortality. In SCD-HeFT, 22% in the ICD group had died at 5 years, and 21% had an appropriate shock.185 However, at 5 years 57% had survived, but had not received an appropriate ICD shock and never used the device.

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