Impact of the Main Implantable Cardioverter-Defibrillator Trials for Primary and Secondary Prevention in Italy: A Survey of the National Activity During the Years 2001–2004

Background: Several trials demonstrated the lifesaving role of implantable cardioverter-defibrillator (ICD) in high-risk groups of patients. Aim of this review was to report the clinical characteristics of patients enrolled in the ICD Registry of the Italian Association of Arrhythmology (AIAC) in the years 2001–2004.
Methods: The Registry collects prospectively 85% of national ICD implantation activity on the basis of European ICD form (EURID).
Results: The number of implanted ICDs in Italy was 2,418 in the year 2001, 3,992 in the year 2002, 5,595 in the year 2003, and 7,190 in the year 2004. The number of ICDs per million of inhabitants was 42.1 in the year 2001 (+11.8% respect to 2000), 70.0 in the year 2002 (+65.1% respect to 2001), 98.3 in the year 2003 (+40.4% respect to 2002), and 125.0 in the year 2004 (+27.2% respect to 2003). The median age was 67 years in the years 2001–2002, 68 years in the years 2003–2004. The main indications during the study were syncope (24.2–14.9%) and cardiac arrest (28.5–17.3%), followed by palpitations and dizzy spells (15.5–17.2%, and 9.4–6.9% of patients, respectively). The use of prophylactic ICD had a fourfold increase in the examined period (5.8% in 2001, 22.9% in 2004). Ventricular tachycardia was the main arrhythmic indication in 44.4–54.6% of cases, ventricular fibrillation in 11.8–18.0%, both in 3.5–6.5%. In the years 2002, 2003, and 2004 single chamber ICDs were implanted in 45.5%, 38.8%, and 33.7% of patients, dual chamber ICDs in 35.1%, 32.3%, and 30.5%, biventricular ICDs in 19.4%, 28.9%, and 34.7%, respectively.
Conclusion: The ICD implantation rate in Italy increased significantly in the period 2001–2004, similarly to the trend in other western countries. The Registry showed an important increase of prophylactic and dual or triple chamber ICDs use.     

Sudden Bradyarrhythmic Death in Patients with the Implantable Cardioverter-Defibrillator: Report of Two Cases

Two cases of sudden bradyarrhythmic death necessitating cardiopulmonary resuscitation in patients with the automatic implantable cardioverter-defibrillator are described. One patient had sudden bradyarrhythmic death while being monitored in the hospital. This patient could not be resuscitated and represents the first reported sudden cardiac death secondary to bradyarrhythmia in a patient with the automatic implantable cardioverter-defibrillator. The second patient had an out-of-hospital sudden death followed by probable cardioverter-defibrillator discharge, leading to complete heart block with escape ventricular rhythm necessitating immediate temporary pacemaker insertion. These cases highlight the need for bradycardia back-up pacing and event monitoring in the newer tachyarrhythmia management devices.     

Implantable Cardioverter-Defibrillator: Patient Selection and Implantation Protocol

L'expérience acquise lors des essais cliniques avec le défibrillateur implantable souligne l'importance d'un bilan physiopathologique précis dans la période préopératoire. Cette évaluation demande une documentation précise de la tachyarrythmie ventriculaire, une évaluation de la possibilité d'autres interventions éventuelles aussi bien que la considération de l'intéraction entre stimulateurs et médicamments antiarrythmiques.     

Clinical Performance of the Implantable Cardioverter-Defibrillator

Le cardioverter-défibrillateur automatique implantable a été implanté chez 276 patients porteurs de tachycardies malignes tenaces avec une survie jusqu'è 50 mois (moyenne = 9.75 mois). Les results de cet appareil sont encourageants. Les risques et les complications associées sont acceptables et ressemblent è ceux des pacemakers. L'accélération des tachycardies ventriculaires ou l'induction de fibrillation ventriculaire sont traitées par le recyclage de l'appareil. Le défibrillateur a réduit la mortalité de ces patients è 2% durant la première année.     

Implantable cardioverter-defibrillators in patients with left ventricular noncompaction

Background: Left ventricular noncompaction (LVNC) is a rare, congenital cardiomyopathy and can be associated with heart failure, embolic events, arrhythmias, and sudden cardiac death. Implantation of implantable cardioverter-defibrillators in these patients is a treatment option, but data on long-term follow-up are limited. The aim of the study was to analyze the clinical outcome of patients with LVNC who were treated with an implantable cardioverter-defibrillator (ICD).
Methods: We conducted a retrospective study on 12 patients (mean age: 45 ± 13 years, range 20–60) with LVNC, who underwent ICD implantation for secondary (n = 8) and primary (n = 4) prevention.
Results: During a median follow-up of 36 months, five patients (42%) presented with appropriate ICD therapy: in four of the eight patients (50%) in whom the ICD was implanted as a secondary prevention and in one of the four patients (25%) for whom the ICD was implanted for primary prevention. In eight patients (66%) supraventricular tachyarrhythmias were documented. Improvement of left ventricular function could be observed in one of two patients with a biventricular ICD.
Conclusions: Potentially life-threatening ventricular tachyarrhythmias may occur in patients with LVNC. ICD therapy may be effective for primary and secondary prevention in these patients. Due to the high prevalence of supraventricular tachyarrhythmias devices with reliable detection enhancements should be considered.     

Mechanism of Death in Patients with the Automatic Implantable Cardioverter Defibrillator

Fifty patients underwent primary implantation of an automatic implantable cardioverter defibrillator between August 1983 and April 1988 and were entered into a long-term surveillance program. There were a total of 14 deaths (28%) in the entire group occurring at a mean of 8.7 months postimplantation. Eleven deaths were cardiac and three were noncardiac (two pneumonia, one leukemia). The group of deceased patients were similar to the survivors in all respects except for a statistically lower ejection fraction (23% vs 32%) at the time of implantation. In addition, 13/14 (93%) of the deceased patients experienced at least one appropriate AICD discharge at a mean of 4.5 months post implantation. Recorded ECGs at the time of death revealed that most of the sudden deaths were due to electromechanical dissociation and not to AICD-treatable arrhythmias. These data suggest therefore that death in AICD patients is usually cardiac, due primarily to low ejection fraction and occurs in patients who have previously received AICD discharges.     

Early Benefit of Implantable Cardioverter Defibrillator Therapy in Patients Waiting for Cardiac Transplantation

The ICD can effectively recognize and treat ventricular arrhythmias that can lead to sudden death. Sudden death is a major problem in patients awaiting heart transplantation. We reviewed our experience with the ICD in patients with malignant ventricular arrhythmias waiting for cardiac transplantation. Nineteen patients were included. Seventeen were men, mean age was 54 ±11 years (range 17–66) and the left ventricular ejection fraction was 22%± 10% (range 9%–46%). After a mean follow–up of 6 ± 5 months (range 1–20 months), 17 patients reached heart transplantation. One patient died and the other is waiting for a transplant. Before transplantation 71 % of patients received an appropriate discharge. The mean time to the first appropriate discharge was 2 ± 2 months (range < 1–6 months), which was significantly shorter than the mean time to first discharge in the other patients (n = 182) receiving a defibrillator in our center (11 ±10 months; range 1–58 months) (P < 0.0004). In conclusion, cardiac transplantation candidates with life–threatening ventricular arrhythmias can effectively be protected against sudden arrhythmic death by ICD. These patients have a high incidence of appropriate shocks occurring very early after implantation.     

The Abuse of Rest as a Therapeutic Agent

Abstract

Sudden cardiac death (SCD) due to ventricular tachyarrhythmias is an important cause of mortality in the United States, 4% of which occurs in patients with structurally normal hearts. At least some arrhythmias are caused by ≥ 1 mutation in 1 of the genes that control electrical conduction through the heart by altering calcium homeostasis or depolarization or repolarization gradients in the ventricle. Although SCD may be the first presentation, patients may often present with symptoms of palpitations or hemodynamic compromise, such as dizziness, seizure, or syncope, particularly following exertion. They may also be made aware of possibly having the condition due to symptoms in other family members. The primary care physician is ideally placed to investigate these symptoms, including detailed clinical and family histories and examining the baseline electrocardiogram. In all inherited cardiac death syndromes, first-degree relatives should be referred to a cardiologist, and should undergo testing appropriate for the condition. While management of patients at risk of SCD largely centers on risk stratification and, if necessary, insertion of an implantable cardioverter-defibrillator, there are a number of other treatments being developed. β-Blockers are often very effective in preventing arrhythmic episodes associated with catecholaminergic polymorphic ventricular tachycardia and some subtypes of long QT syndrome. In certain situations, calcium channel blockers may also be used. Quinidine and isoproterenol can be useful in treating Brugada syndrome. Left cervicothoracic stellectomy may occasionally be used in the treatment of long QT syndrome. As the genetic basis of these diseases becomes known, genetic testing is forming an increasingly important part of diagnosis, and gene-specific therapy is an area under investigation.     

Subpectoral Implantation of ICD Generators: Long-Term Follow-Up

A nonthoracotomy surgical approach using an endocardial electrode and combined implantation of a subcutaneous patch and the implantable cardioverter defibrillator (ICD) generator in a Subpectoral pocket has been described. We report the long-term follow-up results in patients undergoing implantation using this approach. The patient population consisted of 28 patients (22 men and 6 women) with a mean age of 59 ± 12 years. The underlying heart disease consisted of coronary artery disease in 20 patients and dilated cardiomyopathy in 8 patients. Sustained ventricular tachycardia was the mode of presentation in 16 patients and sudden cardiac death in 12 patients. The mean left ventricular ejection fraction was 31%± 6%. The lead system consisted of an 8 French bipolar passive fixation rate sensing lead positioned at the right ventricular apex, an 11 French spring coil electrode positioned at the superior vena cava-right atrial junction (surface area 700 mm²), and submuscular placement of a large patch (surface area 28 cm²) on the anterolateral chest wall near the cardiac apex via a submammary incision. A defibrillation threshold of ≤ 15 joules (J) was required for implantation. This criterion was not satisfied in five patients; thus, a limited thoracotomy was performed via the submammary incision, and the large patch was placed epicardially. The mean R wave amplitude was 12 ± 3 mV, the mean pacing threshold was 1.0 ± 0.5 V at 0.5 msec, and the mean defibrillation threshold was 12.6 ± 3 J. ICD generators implanted were the Ventak-P in 17, PCD-7217 in 5, and the Cadence V-l00 in 6 patients. These patients have been followed for a mean of 14.6 ± 6 months. There was no perioperative mortality, and none of the patients developed an infection during follow-up. Generator migration or significant discomfort requiring ICD repositioning was not observed, although one patient developed an erosion requiring surgical repair.Conclusions: Subpectoral implantation of the ICD generator is feasible and was well tolerated by all patients with an acceptable complication rate (3.5%). As the size of future generation ICDs is reduced, subpectoral implantation may become the preferred approach.     

Sustained polymorphic arrhythmias induced by programmed ventricular stimulation have prognostic value in patients receiving defibrillators

BACKGROUND: Patients with ischemic cardiomyopathy (ICM) who have monomorphic ventricular tachycardia (VT) induced by programmed ventricular stimulation (PVS) are at increased risk of sudden cardiac death (SCD). Among a primary prevention population, the prognostic significance of induced polymorphic ventricular arrhythmias is unknown. METHODS: A total of 105 consecutive patients who received an implantable cardioverter-defibrillator (ICD) for primary prevention of SCD in the setting of ICM and non-sustained VT were retrospectively evaluated. Seventy-five patients (group I) had induction of monomorphic VT and 30 patients (group II) had a sustained ventricular arrhythmia other than monomorphic VT (ventricular flutter, ventricular fibrillation, and polymorphic VT) induced during PVS. RESULTS: Baseline characteristics were similar between group I and group II except for ejection fraction (25% vs. 31%, P = 0.0001) and QRS duration (123 milliseconds vs. 109 milliseconds, P = 0.04). Sixteen of 75 (21.3%) patients in group I and 6 of 30 (20%) patients in group II received appropriate ICD therapy (P = 0.88). Survival free from ICD therapy was similar between groups (P = 0.54). There was a trend toward increased all-cause mortality among patients in group I by Kaplan-Meier analysis (P = 0.08). However, when adjusted for age, EF, and QRS duration mortality was similar (P = 0.45). CONCLUSIONS: There is no difference in rates of appropriate ICD discharge or mortality between patients dichotomized by type of rhythm induced during PVS. These results suggest that patients in this population who have inducible VF or sustained polymorphic VT have similar rates of subsequent clinical ventricular tachyarrhythmias as those with inducible monomorphic VT.     

Use of Electrical Pacing and Automatic Cardioversion Defibrillation for Normal Cardiac Function Recovery

Antitachycardia pacing and low energy cardioversion terminated episodes of ventricular tachycardia and fibrillation and prevented extrasystoles in patients with acute myocardial infarction, coronary artery disease, and long QT syndrome. Low energy automatic defibrillation was used in 25 patients intraoperatively and during the early postoperative period. The Soviet prototype of an implantable cardioverter-defibrillator [ICAR-D] detects the arrhythmia automatically and delivers selectively an electrical impulse to the heart: 2.5–4 J for VT and 15–20 J for VF. In addition, the effectiveness of ICARD was studied in chronical experiments in 10 dogs with follow-up of 4–6 months.     

Actuarial Incidence and Pattern of Occurrence of Shocks Following Implantation of the Automatic Implantable Cardioverter Defibrillator

The actuarial incidence and pattern of occurrence of shocks were analyzed in 65 patients after implantation of the automatic implantable cardioverter defibrillator. During a mean follow-up of 25 +/- 21 months only one patient died suddenly, and this patient had a nonfunctioning device at the time of death. The long-term actuarial risk of death from any cause in the patients who received appropriate shocks was not significantly different than for the entire group. The 1- and 4-year cumulative risk of receiving any shock was 51 +/- 7% and 81 +/- 11%; of receiving an appropriate shock was 33 +/- 7% and 64 +/- 10%; of receiving a spurious shock was 17 +/- 5% and 21 +/- 6%; and of receiving an "indeterminate" shock was 19 +/- 6% and 52 +/- 10%. In 14 patients who were followed for 24 months without receiving an appropriate shock, the actuarial risk of receiving an appropriate shock was 29 +/- 14% during the next 24 months. The mean number of shocks delivered during appropriate episodes was 1.6 +/- 0.9, which was significantly lower than the mean of 4.0 +/- 2.0 shocks during spurious episodes (P less than 0.02). The mean number of shocks during indeterminate episodes was 1.7 +/- 1.5. Our data confirms the efficacy of the implantable defibrillator in preventing sudden death. The majority of patients with this device receive appropriate shocks during long-term follow-up, and the cumulative incidence of appropriate shocks increases steadily for at least 4 years. In contrast, the cumulative incidence of spurious shocks plateaus at about 12 months. Our data suggests that many "indeterminate" shocks actually appear to be appropriate.     

Right Ventricular Dysfunction and the Incidence of Implantable Cardioverter‐Defibrillator Therapies

Introduction: Implantable cardioverter‐defibrillator (ICD) therapy is well established in preventing sudden cardiac death in patients with left ventricular dysfunction. The influence of right ventricular (RV) function on ICD therapy for sudden cardiac death (SCD) is not known. Methods: We retrospectively studied 222 patients receiving an ICD for primary prevention of SCD. Baseline clinical and echocardiographic data were gathered. RV systolic function was qualitatively assessed as normal or abnormal (described as mildly, moderately, or severely reduced). Primary endpoint was combined ICD therapy or death and secondary endpoint was ICD therapy alone. Results: The mean follow‐up was 940 ± 522 days. The mean left ventricular ejection fraction was 0.23 ± 0.07. By Kaplan‐Meier analysis, RV dysfunction was predictive of combined ICD therapy or death when comparing between normal and abnormal RV function (P = 0.008) and among qualitative ranges of RV function (P = 0.012). RV dysfunction was not predictive of ICD therapy alone with either type of classification. After adjusting for clinical covariates, severe RV dysfunction was predictive of the combined endpoint of ICD therapy or death (HR 2.02, 95% CI 1.04–3.92, P = 0.037). Conclusion: Severe RV dysfunction appears to be an independent predictor of the combined endpoint of ICD therapy or death. RV dysfunction does not reliably predict the incidence of ICD therapy alone.     

Brugada syndrome: clinical,genetic, molecular,cellular and ionic aspects

Brugada syndrome, first described as a new clinical entity by Pedro and Josep Brugada in 1992, has attracted great interest because of its high prevalence in many regions of the world and its association with high risk for sudden death. The syndrome has captured the attention of the cardiac electrophysiology community because it serves as a paradigm for our understanding of the role of spatial dispersion of repolarization in the development of cardiac arrhythmias. The past decade has witnessed an exponential rise in the number of reported cases and a striking proliferation of papers serving to define the clinical, genetic, cellular, ionic and molecular aspects of this disease. This brief review summarizes the key clinical and experimental milestones that have brought us to our current understanding and approach to therapy of the Brugada syndrome.     

The role of ion channelopathies in sudden cardiac death: Implications for clinical practice

Abstract

Sudden cardiac death (SCD) following ventricular tachyarrhythmias constitutes an important clinical cause of mortality; 4% of cases may involve ion channel-mediated cellular excitation in structurally normal hearts. Alterations in such processes could disturb action potential conduction, depolarization/ repolarization gradients, or Ca²⁺ homeostasis with potential arrhythmogenic consequences. Although SCD may be the first presentation of arrhythmic syndromes, patients may present to the general physician with symptoms of palpitations or hemodynamic compromise, including dizziness, seizure, or syncope, particularly following exertion. In all inherited cardiac death syndromes, first-degree relatives should be referred to a cardiologist and should undergo testing appropriate for the condition. While management of patients at risk of SCD largely centers on risk stratification and, if necessary, insertion of an implantable cardioverter-defibrillator, there are a number of other, pharmacological, treatments being developed. Furthermore, as the genetic basis of these diseases becomes established, genetic testing will form an increasingly important part of diagnosis, and gene-specific therapy is an area under investigation. This article bridges the gap between molecular medicine and clinical practice by reviewing recent developments in the pathophysiological understanding of SCD, and their implications for the management of patients with these complex diseases.     

Use of the Elective Replacement Indicator in Predicting Time of Automatic Implantable Cardioverter-Defibrillator Battery Depletion

The elective replacement indicator (ERI) of an automatic implantable cardioverter-defibrillator (AICD) is intended to be utilized as strict criteria for the appropriate timing of pulse generator replacement. The manufacturer's newest recommendations suggest replacement within 3 months post-ERI attainment and this applies to both AID-B units and the latest Ventak models currently being implanted with expectations or increased battery longevity. Twelve patients with the AID-B model were prospectively evaluated in regard to (a) time from attainment of ERI to generator exchange and (b) total battery life with respect to the presence or absence of spontaneous discharges. In the overall group, the ERI was reached at 18.9 +/- 3.9 months (mean +/- standard deviation). The time from ERI attainment to generator exchange due to battery depletion was 8.1 +/- 5.1 months, and total battery life was 27.3 +/- 3.1 months. In the group with spontaneous discharges, the duration of time (months) post-ERI to generator exchange was 5.7 +/- 2.6 but was 11.4 +/- 6.1 in the others (P = 0.052); battery life was 25.7 +/- 2.4 and 29.4 +/- 2.7, respectively (P = 0.03). We conclude that the current ERI recommendation seems overly conservative in planning generator exchange for patients without the occurrence of spontaneous discharges and appropriate for patients with such discharges.     

Retroperitoneal Placement of an ICD Generator: A Solution for a Difficult Problem

A woman experienced multiple episodes of pocket erosion after placement of an implantable cardioverter defibrillator culminating in a pocket infection. The device was placed in a retroperitoneal location, and the patient has not developed further episodes of pocket erosion or infection. Interrogation of the device in this position and its subsequent replacement have been straightforward.