Clinical Significance of J Waves in Patients Undergoing Therapeutic Hypothermia for Out-of-Hospital Cardiac Arrest.

BACKGROUND: Hypothermia is associated with the development of J waves. However, little is known about the impact of these electrocardiogram (ECG) findings on the development of ventricular arrhythmias and patient outcomes during therapeutic hypothermia (TH) postresuscitation from out-of-hospital cardiac arrest (OHCA). We investigated the prevalence of J waves in OHCA patients prior to and during TH. Additionally, we explored the incidence of atrial and ventricular arrhythmias and in-hospital mortality for patients with and without J waves either at baseline, during TH, or both. METHODS: We conducted a retrospective analysis of patients who suffered OHCA and underwent TH (goal temperature of 32-34°C). Fifty-nine patients were stratified dependent upon the presence of or the development of J waves on surface ECGs. Descriptive analysis and logistic regression modeling were used to assess the population differences and mortality, respectively, between patients who developed J waves during TH and those who did not. RESULTS: There was no difference in the development of in-hospital atrial or ventricular arrhythmias between patients with J waves present during TH (16%) and those without (17.6%, P = 0.834). Compared to patients without J waves at baseline and during TH, those with J waves present both at baseline and during TH had significantly worse survival (hazard ratio = 12.42, P = 0.046). CONCLUSIONS: While J waves are common ECG findings during TH in patients resuscitated from OHCA, our study demonstrated an increase in mortality for patients with J waves present both at baseline and during TH.

Electrophysiology testing and the National Cardiovascular Data Registry: stimulating, yet inconclusive.

In this issue of PACE, Cheng et al. do an impressive job in evaluating clinical variables associated with electrophysiology studies (EPS) performed within 1 month before implantable cardioverter defibrillator (ICD) placement in 33,786 individuals entered into the National Cardiovascular Data Registry for Implantable Cardioverter Defibrillators (NCDR®-ICD) over a 3-year period. Although of great interest, most of the conclusions are by necessity based on conjecture drawn from observations alone, since the inherent, point-in-time structure of the Registry limits the ability to assess accurate longitudinal clinical correlations and outcomes. The fact is, we really do not know why these patients underwent EPS or how the data from these tests were used. In addition to stimulating speculation on the role of EPS in ICD recipients, the present report should promote caution regarding what conclusions can and should be drawn from the NCDR®-ICD in its present format. As constructed, the Registry provides demographic data and clinical elements up to only a fixed point in time. Hence, the ability to draw conclusions is limited by the abundance of disconnected variables and snapshot quality of data in the NCDR.

Temporal and Global Trends of the Incidence of Sudden Cardiac Death in Hypertrophic Cardiomyopathy.

BACKGROUND: Since the initial clinical description of hypertrophic cardiomyopathy (HCM) over 60 years ago, sudden cardiac death (SCD) has been the most visible and feared complication of HCM. OBJECTIVES: This study sought to characterize the temporal, geographic, and age-related trends of reported SCD rates in adult HCM patients. METHODS: Electronic databases were systematically searched up to November 2021 for studies reporting on SCD event rates in HCM patients. Patients with SCD equivalents (appropriate implantable cardioverter-defibrillator [ICD] shocks and nonfatal cardiac arrests) were not included. A random-effects model was used to pool study estimates calculating the overall incidence rates (IR) for each time-era, geographic region, and age group. We analyzed 2 periods (before vs after 2000, following clinical implementation of ICD in HCM). Following 2000, 5-year intervals were used to demonstrate the temporal change in SCD rates. RESULTS: A total of 98 studies (N = 70,510 patients and 431,407 patient-years) met our inclusion criteria. The overall rate of HCM SCD was 0.43%/y (95% CI: 0.37-0.50%/y; I2 = 75%; SCD events: 1,938; person-years of follow-up: 408,715), with young patients (<18 years of age) demonstrating a >2-fold-risk for sudden death vs adult patients 18-60 years of age (IR: 1.09%; 95% CI: 0.69%-1.73% vs IR: 0.43%; 95% CI: 0.37%-0.50%) (P value for subgroup differences <0.01). Contemporary SCD rates from 2015 to present were 0.32%/y and significantly lower compared with 2000 or earlier (IR: 0.32%; 95% CI: 0.20%-0.52% vs IR: 0.73%; 95% CI: 0.53%-1.02%, respectively). Reported SCD rates for HCM were lowest in North America (IR: 0.28%; 95% CI: 0.18%-0.43%,) and highest in Asia (IR: 0.67%; 95% CI: 0.54%-0.84%). CONCLUSIONS: Contemporary HCM-related SCD rates are low (0.32%/y) representing a 2-fold decrease compared with prior treatment eras. Young HCM patients are at the highest risk. The maturation of SCD risk stratification strategies and the application of primary prevention ICD to HCM are likely responsible for the notable decline over time in SCD events. In addition, worldwide geographic disparities in SCD rates were evident, underscoring the need to increase access to SCD prevention treatment for all HCM patients.

Importance of newer cardiac magnetic resonance-based risk markers for sudden death prevention in hypertrophic cardiomyopathy: An international multicenter study.

BACKGROUND: The sudden death (SD) risk stratification algorithm in hypertrophic cardiomyopathy (HCM) has evolved, underscored recently by novel cardiac magnetic resonance (CMR)-based risk markers (left ventricular apical aneurysm, extensive late gadolinium enhancement, and end-stage disease with systolic dysfunction) incorporated into the 2020 American Heart Association (AHA)/American College of Cardiology (ACC) HCM guidelines. OBJECTIVE: The purpose of this study was to assess the specific impact of newer, predominantly CMR-based risk markers in a large multicenter HCM population that underwent primary prevention implantable cardioverter-defibrillator (ICD) implants. METHODS: Longitudinal study of 1149 consecutive HCM patients from 6 North American and European HCM centers prospectively judged to be at high SD risk based on ≥1 AHA/ACC individual risk markers and prophylactically implanted with an ICD was performed. European Society of Cardiology (ESC) risk score was retrospectively analyzed with respect to the known clinical outcome. RESULTS: Of 1149 patients with an ICD, 162 (14%) experienced device therapy terminating ventricular tachycardia/ventricular fibrillation 4.6 ± 4.2 years after implant. CMR-based markers solely or in combination led to ICD implantation in 49 of the 162 patients (30%) experiencing device therapy. Particularly low ESC scores (<4%/5 years) would have excluded an ESC ICD recommendation for 67 patients who nevertheless experienced appropriate ICD therapy, including 26 with the CMR-based risk markers not part of the ESC formula. CONCLUSION: Identification and incorporation of novel guideline-supported CMR-based risk markers enhance selection of HCM patients for SD prevention with ICDs. Absence of CMR-based markers from the ESC risk score accounts, in part, for it not identifying many HCM patients with SD events. These data support inclusion of CMR as a routine part of HCM patient evaluation and risk stratification.

Utility and safety of axillo-subclavian venous imaging with carbon dioxide (CO) prior to chronic lead system revisions.

BACKGROUND: Prior to attempting placement of one or more electrodes to revise existing rhythm control devices, patency of the central veins should be documented, in view of a high incidence of significant chronic occlusions. Since iodinated contrast venography may be contraindicated in select situations, imaging of the axillo-subclavian venous system with gaseous carbon dioxide (CO(2)) was evaluated prospectively in 23 consecutive individuals who were considered for revision of previously implanted pacemaker or automatic cardioverter defibrillator lead systems. METHODS: Approximately 20 mL of CO(2) were manually infused via CO(2) primed injection tubing into a vein at or above the level of the antecubital fossa ipsilateral to the side of prior lead placements. Digital subtraction imaging over the axillo-subclavian region, lower neck, and mediastinum was performed. Formal interpretation was obtained from one of three interventional radiologists and at least one electrophysiologist. RESULTS: Significant venous occlusions were identified in five (22%) patients. Vascular access utilized for the subsequent 18 revisions performed included the imaged patent ipsilateral vein in 14 patients and the contralateral, right-sided subclavian venous system in three patients. One patient required epicardial left ventricular lead placement. There were no complications from venography. CONCLUSIONS: Axillo-subclavian venography with gaseous CO(2) in patients undergoing pacemaker or implantable cardioverter defibrillator lead revisions is feasible and safe when use of iodinated dye is contraindicated. This technique should be employed in patients with azotemia, dye contrast allergies, or significant inflammation in the vicinity of the intravenous line insertion.

Ventricular arrhythmia inducibility predicts subsequent ICD activation in nonischemic cardiomyopathy patients: a DEFINITE substudy.

OBJECTIVES: We evaluated whether electrophysiologic (EP) inducibility predicts the subsequent occurrence of spontaneous ventricular tachycardia (VT) or ventricular fibrillation (VF) in the Defibrillators in Nonischemic Cardiomyopathy Treatment Evaluation (DEFINITE) trial. BACKGROUND: Inducibility of ventricular arrhythmias has been widely used as a risk marker to select implantable cardioverter defibrillator (ICD) candidates, but is believed not to be predictive in nonischemic cardiomyopathy patients. METHODS: In DEFINITE, patients randomized to the ICD arm, but not the conventional arm, underwent noninvasive EP testing via the ICD shortly after ICD implantation using up to three extrastimuli at three cycle lengths plus burst pacing. Inducibility was defined as monomorphic or polymorphic VT or VF lasting 15 seconds. Patients were followed for a median of 29 +/- 14 months (interquartile range = 2-41). An independent committee, blinded to inducibility status, characterized the rhythm triggering ICD shocks. RESULTS: Inducibility, found in 29 of 204 patients (VT in 13, VF in 16), was associated with diabetes (41.4% vs 20.6%, P = 0.014) and a slightly higher ejection fraction (23.2 +/- 5.9 vs 20.5 +/- 5.7, P = 0.021). In follow-up, 34.5% of the inducible group (10 of 29) experienced ICD therapy for VT or VF or arrhythmic death versus 12.0% (21 of 175) noninducible patients (hazard ratio = 2.60, P = 0.014). CONCLUSIONS: In DEFINITE patients, inducibility of either VT or VF was associated with an increased likelihood of subsequent ICD therapy for VT or VF, and should be one factor considered in risk stratifying nonischemic cardiomyopathy patients.

Clinical Course and Quality of Life in High-Risk Patients With Hypertrophic Cardiomyopathy and Implantable Cardioverter-Defibrillators.

BACKGROUND: High-risk patients with hypertrophic cardiomyopathy (HCM) are identified by contemporary risk stratification and effectively treated with implantable cardioverter-defibrillators (ICDs). However, long-term HCM clinical course after ICD therapy for ventricular tachyarrhythmias is incompletely understood. METHODS AND RESULTS: Cohort of 486 high-risk HCM patients with ICDs was assembled from 8 international centers. Clinical course and device interventions were addressed, and survey questionnaires assessed patient anxiety level and psychological well-being related to ICD therapy. Of 486 patients, 94 (19%) experienced appropriate ICD interventions terminating ventricular tachycardia/ventricular fibrillation, 3.7% per year for primary prevention, over 6.4±4.7 years. Of 94 patients, 87 were asymptomatic or only mildly symptomatic at the time of appropriate ICD interventions; 74 of these 87 (85%) remained in classes I/II without significant change in clinical status over the subsequent 5.9±4.9 years (up to 22). Among the 94 patients, there was one sudden death (caused by device failure; 1.1%); 3 patients died from other HCM-related processes unrelated to arrhythmic risk (eg, end-stage heart failure). Post-ICD intervention, freedom from HCM mortality was 100%, 97%, and 92% at 1, 5, and 10 years, distinctly lower than in ischemic or nonischemic cardiomyopathy ICD trials. HCM patients with ICD interventions reported heightened anxiety in expectation of future shocks, but with intact general psychological well-being and quality of life. CONCLUSIONS: In HCM, unlike ischemic heart disease, prevention of sudden death with ICD therapy is unassociated with significant increase in cardiovascular morbidity or mortality, or transformation to heart failure deterioration. ICD therapy does not substantially impair overall psychological and physical well-being.

Are implantable cardioverter defibrillator shocks a surrogate for sudden cardiac death in patients with nonischemic cardiomyopathy?

BACKGROUND: Ventricular tachyarrhythmias long enough to cause implantable cardioverter defibrillator (ICD) shocks are generally thought to progress to cardiac arrest. In previous ICD trials, shocks have been considered an appropriate surrogate for sudden cardiac death (SCD) because the number of shocks has been thought to be equivalent to the mortality excess in patients without ICDs. The practice of equating ICD shocks with mortality is controversial and has not been validated critically. METHODS AND RESULTS: The Defibrillators in Non-Ischemic Cardiomyopathy Treatment Evaluation (DEFINITE) trial was a prospective, randomized, multicenter trial of ICD therapy in 458 patients with nonischemic cardiomyopathy. Patients were randomized to receive standard medical therapy (STD) or STD plus an ICD. Shock electrograms were reviewed, and the cause of death was evaluated by a separate blinded events committee. There were 15 SCD or cardiac arrests in the STD group and only 3 in the ICD arm. In contrast, of the 229 patients randomized to an ICD, 33 received 70 appropriate ICD shocks. Patients in the ICD arm were more likely to have an arrhythmic event (ICD shock plus SCD) than patients in the STD arm (hazard ratio 2.12, 95% CI 1.153 to 3.893, P=0.013). The number of arrhythmic events when one includes syncope as a potential arrhythmic event was similar in both groups (hazard ratio 1.20, 95% CI 0.774 to 1.865, P=0.414). Approximately the same number of total events was noted in each arm when we compared syncope plus SCD/cardiac arrest in the STD arm with SCD plus ICD shocks plus syncope in the ICD arm. CONCLUSIONS: Appropriate ICD shocks occur more frequently than SCD in patients with nonischemic cardiomyopathy. This suggests that episodes of nonsustained ventricular tachycardia frequently terminate spontaneously in such patients.

Implantable cardioverter-defibrillators and prevention of sudden cardiac death in hypertrophic cardiomyopathy.

CONTEXT: Recently, the implantable cardioverter-defibrillator (ICD) has been promoted for prevention of sudden death in hypertrophic cardiomyopathy (HCM). However, the effectiveness and appropriate selection of patients for this therapy is incompletely resolved. OBJECTIVE: To study the relationship between clinical risk profile and incidence and efficacy of ICD intervention in HCM. DESIGN, SETTING, AND PATIENTS: Multicenter registry study of ICDs implanted between 1986 and 2003 in 506 unrelated patients with HCM. Patients were judged to be at high risk for sudden death; had received ICDs; underwent evaluation at 42 referral and nonreferral institutions in the United States, Europe, and Australia; and had a mean follow-up of 3.7 (SD, 2.8) years. Measured risk factors for sudden death included family history of sudden death, massive left ventricular hypertrophy, nonsustained ventricular tachycardia on Holter monitoring, and unexplained prior syncope. MAIN OUTCOME MEASURE: Appropriate ICD intervention terminating ventricular tachycardia or fibrillation. RESULTS: The 506 patients were predominately young (mean age, 42 [SD, 17] years) at implantation, and most (439 [87%]) had no or only mildly limiting symptoms. ICD interventions appropriately terminated ventricular tachycardia/fibrillation in 103 patients (20%). Intervention rates were 10.6% per year for secondary prevention after cardiac arrest (5-year cumulative probability, 39% [SD, 5%]), and 3.6% per year for primary prevention (5-year probability, 17% [SD, 2%]). Time to first appropriate discharge was up to 10 years, with a 27% (SD, 7%) probability 5 years or more after implantation. For primary prevention, 18 of the 51 patients with appropriate ICD interventions (35%) had undergone implantation for only a single risk factor; likelihood of appropriate discharge was similar in patients with 1, 2, or 3 or more risk markers (3.83, 2.65, and 4.82 per 100 person-years, respectively; P = .77). The single sudden death due to an arrhythmia (in the absence of advanced heart failure) resulted from ICD malfunction. ICD complications included inappropriate shocks in 136 patients (27%). CONCLUSIONS: In a high-risk HCM cohort, ICD interventions for life-threatening ventricular tachyarrhythmias were frequent and highly effective in restoring normal rhythm. An important proportion of ICD discharges occurred in primary prevention patients who had undergone implantation for a single risk factor. Therefore, a single marker of high risk for sudden death may be sufficient to justify consideration for prophylactic defibrillator implantation in selected patients with HCM.

Relation of 12-lead electrocardiogram patterns to implanted defibrillator-terminated ventricular tachyarrhythmias in hypertrophic cardiomyopathy.

Electrocardiographic (ECG) abnormalities are common in hypertrophic cardiomyopathy (HC) and have been associated with the distribution of left ventricular hypertrophy and myocardial fibrosis. Such abnormalities may predispose patients to electrophysiologic instability, ventricular arrhythmias, and sudden cardiac death (SCD). We studied 330 patients with HC who were judged clinically to be at high risk for SCD and therefore received automatic implantable cardioverter-defibrillators (ICDs). Surface 12-lead electrocardiograms acquired at the time of ICD implantation were analyzed and the ECG characteristics of patients with appropriate device interventions for ventricular tachycardia and fibrillation were compared to those patients without appropriate device interventions. The 330 patients were followed for 3.7 +/- 3.0 years after implantation and 57 patients (17%) had appropriate discharges. No differences in the ECG characteristics of patients with and without appropriate device interventions were identified. Markedly increased ECG voltages, QRS duration, left or rightward QRS axis, abnormal Q waves, and QTc or QT dispersion were not associated with appropriate ICD discharge. Conversely, normal electrocardiograms and electrocardiograms normal except for a repolarization abnormality in only 1 anatomic distribution were not associated with freedom from ICD discharge. Moreover, no combination of ECG variables was associated with the likelihood of an appropriate ICD discharge. In conclusion, in a cohort of patients with HC selected because of their high risk for SCD, 12-lead surface electrocardiogram did not predict subsequent appropriate ICD intervention for ventricular tachyarrhythmias and was not useful in risk stratification for sudden death.

Safety and efficacy of transvenous high-voltage implantable cardioverter-defibrillator leads in high-risk hypertrophic cardiomyopathy patients.

BACKGROUND: The implantable cardioverter-defibrillator (ICD) prevents sudden cardiac death in high-risk patients with hypertrophic cardiomyopathy (HCM). However, recently concerns have been raised regarding the performance of transvenous high-voltage ICD leads (HVL) in this population. OBJECTIVE: The purpose of this retrospective, multicenter study was to assess the safety and efficacy of HVL in high-risk HCM patients. METHODS: The study population consisted of HCM patients who received HVL ICDs and were subsequently followed at seven centers in the United States. Kaplan-Meier survival rates were calculated for HVL and patients. HVL failure was a malfunction caused by a noniatrogenic defect. RESULTS: Between 1992 and 2007, 324 HCM patients (mean age 47 +/- 16 years) received 343 HVL from three major manufacturers. The average HVL implant duration was 3.3 +/- 2.8 years. Overall, the HVL failure rate was 1.4%/year. However, two models (Sprint Fidelis and Transvene, Medtronic, Inc.) accounted for 60% of HVL failures. Survival probabilities for HVL and patients at 10 years were 93% and 91%, respectively. No deaths or serious injuries were reported, although inappropriate shocks occurred in 12% of cases. CONCLUSIONS: This multicenter experience shows that HVLs are safe and effective in high-risk HCM patients. However, differences in failure rates were found between lead models.

Inappropriate defibrillator shocks from depolarization--repolarization mismatch in a patient with hypertrophy cardiomyopathy.

Despite wide use of dedicated bipolar sensing electrodes in implantable cardioverter-defibrillator (ICD) systems, oversensing occasionally occurs, leading to unwarranted shocks or antitachycardia pacing. This case report highlights an individual with hypertrophic cardiomyopathy (HCM) who experienced inappropriate shocks from oversensing of repolarization electrograms (T-waves). During the implantation procedure, no excessive T-wave amplitudes were detected during sinus rhythm, ventricular pacing, or induced ventricular fibrillation. T-wave oversensing leading to shocks only developed after maturation of the lead-tissue interface. An adequate safety margin for discrimination between ventricular electrograms and T-waves could not be assured. Thus, insertion of a new dedicated pacing-sensing electrode was required. The degree to which intracardiac repolarization signals may be heightened in patients with HCM has not been investigated systematically. However, a relative decrease in the ventricular electrogram amplitude without a concomitant decline of the intracardiac T-wave amplitude appears to have led to the problem in this patient. Special caution in technique and device selection with a particular emphasis on T-wave sensing may be prudent when ICDs are implanted in individuals with HCM. Additional programmable variables may also be beneficial in such cases.