Idiopathic polymorphic ventricular tachycardia with normal QT interval in a structurally normal heart

Polymorphic ventricular tachycardia (PVT) is a life-threatening arrhythmia that is typically related to long QT syndrome, organic heart disease, electrolyte abnormalities, cardiotoxic drugs, or adrenergic stimulation. A review of the literature reveals that PVT with normal QT interval and without underlying cause is quite rare. We report a case of idiopathic spontaneous PVT with structurally normal heart and without electrolyte abnormalities, drug reactions, or evidence of catecholamine induced arrhythmia. We also review the literature on the electrocardiographic characteristics and management of idiopathic PVT.     

Circadian variations in the occurrence of ventricular tachyarrhythmias in patients with implantable cardioverter defibrillators

A circadian distribution has been demonstrated in episodes of sudden cardiac death, acute myocardial infarction, ventricular premature complexes, heart rate variability, and ventricular tachyarrhythmias. The aim of this study was to evaluate the circadian distribution of ventricular tachyarrhythmia episodes in a population of ICD patients. Data were gathered from 72 patients (55 men, 17 women; mean age 62.7 +/- 12.2 years, mean LVEF 0.0037 +/- 0.0011) with ICDs implanted for standard indications. Patients were followed every 3 months over a mean period of 21 +/- 12.8 months. At each examination, symptoms at arrhythmia onset and perception of ICD therapy were recorded, and the ICD memory was interrogated. During follow-up, 1,023 episodes' of malignant ventricular arrhythmias were detected and effectively terminated, 506 of which were fully analyzed. A morning peak in ventricular tachyarrhythmias was demonstrated between 7:00 and 11:00 AM, and an afternoon peak between 6:00 and 7:00 PM. A significantly lower occurrence of VT was observed at 1:00 AM and between 4:00 and 6:00 AM. A circadian distribution in the occurrence of ventricular tachycardias was found. The three striking features of the data are: the early morning peak (about three hours after waking up), relatively stable incidence throughout waking hours, and decline in incidence in the previous period.     

Spurious redetection of sinus rhythm by an implantable cardioverter defibrillator during spontaneous ventricular fibrillation

Implantable cardioverter defibrillator undersensing leading to delayed or aborted therapy delivery has been reported with induced arrhythmias and following failed defibrillator shocks. We describe a case in which spurious redetection of sinus rhythm during a spontaneous episode of ventricular fibrillation resulted in aborted device therapy.     

Electrophysiologic study-guided therapy with sotalol for life-threatening ventricular tachyarrhythmias

The aim of this study was to investigate the long-term efficacy and safety of electrophysiologic study (EPS)-guided sotalol administration combined with implantable cardioverter defibrillators (ICD) for ventricular tachyarrhythmias (VTA). This study enrolled 92 patients with both structural heart disease and sustained VTA. Sotalol was administered to 57 patients, and its efficacy was assessed by EPS. Long-term treatment was continued in combination with ICD in 31 patients (57%) whose VTA was no longer inducible (responder group) and in 16 patients whose VTA remained inducible (nonresponder group). The long-term outcomes were compared among the responder group, the nonresponder group, and 35 ICD recipients untreated with antiarrhythmic drugs (ICD-only group). During a mean follow-up of 44 +/- 33 months, the recurrence of VTA was not significantly different between all patients treated with sotalol (30%) and patients in the ICD-only group (46%). However, the recurrence of VTA was significantly lower in the responder (13%) than in the nonresponder (63%) or the ICD-only groups (46%). There was no significant difference in VTA recurrence between the nonresponder and the ICD-only groups. One patient each in the responder and the ICD-only groups died suddenly, and all-cause mortality was similar in the three groups. The incidence of inappropriate ICD discharges was less in the sotalol than in the ICD-only groups. No patient had to discontinue long-term sotalol treatment because of the adverse effects. In conclusion, sotalol reduced VTA recurrence in the responding patients and inappropriate ICD discharge. EPS may predict the efficacy of sotalol for VTA recurrence.     

Implantable defibrillator use for de novo ventricular tachyarrhythmias encountered after cardiac surgery

De novo postoperative life-threatening ventricular arrhythmias are poorly understood. Long-term benefits of, and need for, treatment is uncertain. To assess the therapeutic advantage of ICD to manage new-onset, life-threatening ventricular tachyarrhythmias after cardiac surgery. Patients included were those with an ICD implanted for de novo life-threatening ventricular tachyarrhythmias encountered 48 hours or more after cardiac surgery. Primary endpoints were total survival, time to first ICD therapy, and appropriateness of ICD therapy. Mean projected survival and projected time to first ICD therapy were calculated by the Kaplan-Meier method. Twenty-seven postoperative patients (left ventricular ejection fraction 0.22 +/- 0.07) were followed for 26 +/- 17.6 months. The index arrhythmia was sustained monomorphic ventricular tachycardia in 17 (63%) and ventricular fibrillation in 10 (37%). Electrophysiological study was positive in 22 (81%) of 27. Total survival and mean projected survival after ICD implant were 22 (81%) of 27 and 25.6 months, respectively, to end of follow-up. The majority received ICD therapy (21/27 [78%]), 20 (74%) of 27 receiving appropriate therapy. The mean time to first ICD therapy and mean projected time to first ICD therapy was 5.6 +/- 7.8 months and 10.5 months, respectively. De novo postoperative ventricular arrhythmias are associated with a high probability of late recurrence. The ICD is useful for these patients.     

Levofloxacin induced polymorphic ventricular tachycardia with normal QT interval

Polymorphic ventricular tachycardia (PVT) is a form of ventricular tachycardia characterized by QRS complexes that seem to change direction during the tachycardia. If associated with a prolonged QT interval, it is called torsades de pointes. In the absence of a congenital long QT syndrome, torsades is seen with certain drugs such as antiarrhythmic agents (Class IA, IC, III), psychotropic medications, antidepressants, antihistamines, and electrolyte disturbances. We report the first case of polymorphic ventricular tachycardia with normal QT interval associated with the oral use of levofloxacin in the absence of other etiologies known to cause these arrhythmias.     

Impact of Dual Chamber Pacing on the Incidence of Atrial and Ventricular Tachyarrhythmias in Recipients of Implantable Cardioverter Defibrillators

Recent observations suggest that frequent dual-chamber pacing in recipients of implantable cardioverter defibrillators (ICD) may adversely influence clinical outcomes. This prospective, multicenter study examined the relationship between the frequency of atrial (%AP) and ventricular pacing (%VP) and the incidence of atrial (AT) and/or ventricular tachyarrhythmias (VT) in a standard ICD population. A total of 141 consecutive patients with primary and secondary ICD indications were studied. Continuous arrhythmia detection with a dual-chamber ICD revealed paroxysmal AT in 60 (43%) and VT in 72 (51%) patients within 6 months of device implantation. Far-field oversensing of ventricular signals occurred in 13% of all "atrial tachy response" mode switches. Without adjustment for covariates, a higher %AP was associated with an increased incidence of AT (P < 0.05). However, this association remained only weakly significant after adjustment for covariates using a multivariate model. High New York heart failure functional classes correlated significantly with AT (P = 0.02) and VT (P = 0.007). Rate-modulated pacing, programmed in 1/3 of patients, correlated with occurrence of AT (P = 0.006), but not with occurrence of VT. With respect to dual-chamber pacing, a %AP ≥ 48% combined with a %VP > 40% was associated with an increased probability for VT. In conclusion, AT and VT occurred frequently within 6 months after dual-chamber ICD implantation. High rates of DDD/R stimulation were associated with a trend toward higher incidence of AT, VT, or both.     

Distinct clinical features in the recipients of the implantable cardioverter defibrillator in Taiwan: a multicenter registry study

Little information about the ICD is available from the Asian Pacific region. The purpose of this study was to characterize the clinical features in ICD patients in Taiwan and to compare these features with those in patients in the Western populations, mainly the Canadian Implantable Defibrillator Study (CIDS), the Antiarrhythmics versus Implantable Defibrillator (AVID) trial, and the Cardiac Arrest Study Hamburg (CASH) trial. From February 1995 to October 2001, 101 ICDs were implanted in 92 patients (78 [84%] men) in 12 hospitals. Clinical presentations included sudden cardiac death due to VF/VT in 35 (38%) patients, syncopal VT in 25 (27%), drug refractory nonsyncopal VT in 27 (29%), and unexplained syncope with inducible sustained VT/VF in 5 (6%). The mean age was significantly younger than that in CIDS or AVID (59 +/- 16 vs 63 +/- 9 years in CIDS, P = 0.02; vs 65 +/- 11 years in AVID, P < 0.001), but was comparable to that in CASH (59 +/- 16 vs 58 +/- 11 years in CASH, P = 0.75). The mean LVEF was significantly higher than that in CIDS or AVID (48 +/- 19% vs 34 +/- 15% in CIDS, P < 0.001; vs 32 +/- 13% in AVID, P < 0.001), but was comparable to that in CASH (48 +/- 19 vs 46 +/- 19% in CIDS, P = 0.83). The ICD patients in the current study also showed a higher incidence of normal heart (23 vs 4% in CIDS, P < 0.001; vs 3% in AVID, P < 0.001; vs 9% in CASH, P < 0.001) and cardiomyopathy (41% vs 10% in CIDS, P < 0.001; vs 15% in AVID, P < 0.001; vs 11% in CASH, P < 0.001), but a lower incidence of coronary artery disease (29% vs 83% in CIDS, P < 0.001; vs 82% in AVID, P < 0.001; vs 73% in CASH, P < 0.001). During a mean follow-up of 28 +/- 24 months, 13 (14%) patients died. Older age was the only factor associated with poorer survival after ICD implantation. Forty-seven (51%) patients received appropriate ICD discharges during follow-up. History of prior myocardial infarction was the only factor associated with an earlier first appropriate ICD discharge and LVEF < 0.35 the only factor associated with subsequent poorer survival after the first ICD discharge. In conclusion, this study demonstrated many distinct clinical features in our ICD population that were different from those in the Western populations.     

Antitachycardia pacing for spontaneous rapid ventricular tachycardia in patients with prophylactic cardioverter-defibrillator therapy

AIMS: Antitachycardia pacing (ATP) has not routinely been used in patients who received implantable cardioverter defibrillators (ICDs) for primary prevention of sudden death. This study investigated the efficacy of empirical ATP to terminate rapid ventricular tachycardia (VT) in heart failure patients with prophylactic ICD therapy. METHODS AND RESULTS: Ninety-three patients with a mean left ventricular ejection fraction of 22 +/- 7% (range: 9-35%) due to nonischemic or ischemic cardiomyopathy received prophylactic ICDs with empiric ATP. At least 2 ATP sequences with 6-pulse burst pacing trains at 81% of VT cycle length (CL) were programmed in one or two VT zones for CL below 335 +/- 23 ms and above 253 +/- 18 ms. Ventricular flutter and fibrillation (VF) with CL below 253 +/- 18 ms were treated in a separate VF zone with ICD shocks without preceding ATP attempts. During 38 +/- 27 months follow-up, 339 spontaneous ventricular tachyarrhythmias occurred in 36 of 93 study patients (39%). A total of 232 VT episodes, mean CL 293 +/- 22 ms, triggered ATP in 25 of 36 patients with ICD interventions (69%). ATP terminated 199 of 232 VT episodes (86%) with a mean CL of 294 +/- 23 ms in 23 of 25 patients (88%) who received ATP therapy. ATP failed to terminate or accelerated 33 of 232 VT episodes (14%) with a mean CL of 287 +/- 19 ms in 12 of 25 patients (48%) who received ATP therapy. CONCLUSIONS: Painfree termination of rapid VT with empirical ATP is common in heart failure patients with prophylactic ICD therapy. The occasional inability of empiric ATP to terminate rapid VT in almost 50% of patients who receive ATP for rapid VT warrants restrictive ICD programming with regard to the number of ATP attempts in order to avoid syncope before VT termination occurs.     

Importance of initiation pattern of polymorphic ventricular tachycardia in patients with implantable cardioverter defibrillators

BACKGROUND: Stored intracardiac electrograms (ICEGs) are helpful in understanding the initiation mechanisms of sustained ventricular arrhythmias and in determining the appropriateness of the therapy delivered by implantable cardioverter defibrillators (ICDs). AIM: We investigated the initiation pattern of sustained polymorphic ventricular tachycardia (PVT) and the features of the therapy delivered by ICDs. METHODS: Sixty-six patients (mean age of 67 +/- 8 years) with 97 stored ICEGs showing PVT were evaluated. Cardiovascular diagnosis included coronary artery disease in 72.7% of the patients. The average left ventricular ejection fraction was 33+/-6%. RESULTS: Nonsudden onset episodes were more common than sudden onset episodes (63 episodes, 65% vs 34 episodes, 35%, P < 0.001). More PVT episodes were required multiple shock delivery if they had nonsudden onset initiation (28.6% vs 23.6%, P < 0.01). The mean shock energy delivered for arrhythmia termination was higher in PVT with nonsudden onset (20 +/- 4 vs 14 +/- 5 J, P < 0.01). CONCLUSIONS: The stored ICEGs demonstrate that PVT is most often preceded by ventricular ectopy. To be reverted, nonsudden onset episodes require higher levels of shock energy and more frequently multiple shock achievements than sudden onset episodes.     

Improvement of Quality of Life by Means of Antitachy Pacing: From PainFREE to the ADVANCE-D Trial

Background: Implantable cardioverter-defibrillators (ICD) can terminate ventricular tachyarrhythmias with shocks (painful) or antitachycardia pacing (painless). According to the results of the Pacing Fast VT Reduces Shock ThErapies Trials, antitachycardia pacing (ATP) can avoid painful shocks and also increase device longevity. The purpose of the ADVANCE-D (Atp DeliVery for PAiNless ICD ThErapy) study is to determine the most appropriate ventricular tachycardia (VT) therapy, so as to optimize painless therapy for life-threatening arrhythmias.
Methods and Results: The ADVANCE-D is a prospective, multicenter, parallel, two-arm randomized study designed to evaluate the efficacy of two different sequences of ATP therapies (burst 15 pulses, 88%, vs burst 8 pulses, 88%), during an episode of spontaneous arrhythmia classified as fast VT (FVT) in patients with a Class I or IIA indication for ICD implantation (single and dual chamber devices). The primary endpoint is to compare the efficacy of two ATP therapies for FVT episodes. The study will enroll a minimum of 900 patients within 2 years, followed-up for 12 months. The investigation is expected to be completed in 2007.
Conclusions: The ADVANCE-D trial is the first large randomized clinical investigation aimed to evaluate optimal programming and efficacy of ATP.     

Bridging a Temporary High Risk of Sudden Arrhythmic Death. Experience with the Wearable Cardioverter Defibrillator (WCD)

The implantable cardioverter defibrillator (ICD) is able to reduce sudden arrhythmic death in patients who are considered to be at high risk. However, the arrhythmic risk may be increased only temporarily as long as the proarrhythmic conditions persist, left ventricular ejection fraction remains low, or heart failure prevails. The wearable cardioverter defibrillator (WCD) represents an alternative approach to prevent sudden arrhythmic death until either ICD implantation is clearly indicated or the arrhythmic risk is considered significantly lower or even absent. The WCD is also indicated for interrupted protection by an already implanted ICD, temporary inability to implant an ICD, and lastly refusal of an indicated ICD by the patient. The WCD is not an alternative to the ICD, but a device that may contribute to better selection of patients for ICD therapy. The WCD has the characteristics of an ICD, but does not need to be implanted, and it has similarities with an external defibrillator, but does not require a bystander to apply lifesaving shocks when necessary. The WCD was introduced into clinical practice about 8 years ago, and indications for its use are currently expanding. This article describes the technological aspects of the WCD, discusses current indications for its use, and reviews the clinical studies with the WCD. Additionally, data are reported on the clinical experience with the WCD based on 354 patients from Germany hospitalized between 2000 and 2008 who wore the WCD for a mean of 3 months. (PACE 2010; 33:353–367)     

Long QT Syndrome and Dilated Cardiomyopathy with SCN5A p.R1193Q Polymorphism: Cardioverter-Defibrillator Implantation at 27 Months

Cardiac sodium channel dysfunction associated with the SCN5A gene presents with mixed phenotypes, including long QT syndrome type 3, sinus node dysfunction, and dilated cardiomyopathy (DCM). We report a Korean case of an overlap syndrome of cardiac sodium channelopathy with SCN5A p.R1193Q polymorphism, treated by the placement of an intrapericardial implantable cardioverter-defibrillator (ICD) at the age of 27 months. Although the patient received two appropriate life-saving shocks for ventricular fibrillations, he eventually died of DCM progression. However, this case shows that intrapericardial ICD implantation is feasible in young children with a high risk for sudden cardiac death. (PACE 2012; 35:e243-e246).     

Ventricular rate stabilization for the prevention of pause dependent ventricular tachyarrhythmias: results from a prospective study in 309 ICD recipients

Reviews of stored electrograms from ICDs revealed a 5-30% incidence of short-long-short intervals preceding the onset of recurrent ventricular tachyarrhythmias. Rate stabilization by dedicated antibradycardia pacing algorithms has, therefore, been suggested to prevent onset of pause dependent tachyarrhythmias. However, the clinical efficacy of this approach has not been studied systematically. In a prospective multicenter crossover study, patients were randomized to activation or deactivation of an implemented ventricular rate stabilization algorithm (VRS) after first implant of a dual chamber ICD. After 3 months, all patients were crossed over to the alternate programming. The rate of appropriate spontaneous VA episodes was compared between VRS On and VRS Off. Stored electrograms were reviewed for evaluation of the mode of onset of tachyarrhythmias. Overall efficacy analysis was based on 309 patients enrolled in the study. Forty percent (124/309) of the patients experienced 4,973 VA episodes. Based on an intention-to-treat analysis, VRS Off and On arrhythmia incidence was 10.2 and 6.6 normalized to 3 months, respectively (risk reduction 35%; P = 0.18) On an on-treatment basis, a reduction from 9.0 episodes to 8.1 episodes (10% risk reduction, P = 0.24) was seen. In an extended Cox model adjusting for confounding variables, the relative risk for recurrent episodes was 0.92 during VRS On compared to Off (95% CI: 0.58-1.48; P = 0.74). During VRS Off, pause dependent onset was documented in only 36 (8%) of 427 visually analyzed episodes. There was no significant reduction in the incidence of recurrent ventricular tachyarrhythmias with VRS On compared to the Off programming in this prospective study.     

Discriminatory therapy for very fast ventricular tachycardia in patients with implantable cardioverter defibrillators

Objectives: We assessed the efficacy of antitachycardia pacing (ATP) and low-energy (5J) shock for very fast ventricular tachycardia (VFVT), cycle length 200–250 ms, in patients with implantable cardioverter defibrillators (ICDs).
Methods and Results: One hundred and fifty-two consecutive patients with standard indications for ICD therapy were enrolled. Before discharge from the hospital each patient had an electrophysiological study (EPS) performed through the device, to assess the efficacy of ATP and low-joule shock at terminating VFVT. Initial therapy for VFVT consisted of three bursts of ATP followed by low-energy shock, and high-energy shocks as required. The mean age of enrolled patients was 63 ± 13 years, and the mean left ventricular ejection fraction (LVEF) was 31 ± 13%. During the predischarge EPS, a total of 125 VT episodes were induced in 64 patients. In patients with VFVT, the success rate of ATP was 30% (14/46), the acceleration rate was 26% (12/46), and the success rate of low-energy shock was 86% (25/29). In patients with fast ventricular tachycardia (FVT), cycle lengths 251–320 ms, the success rate of ATP was 62% (24/39), the acceleration rate was 18% (7/39), and the success rate of low-energy shock was 94% (17/18).
Conclusions: This study has demonstrated for the first time that ATP and low-energy shock are effective, as an alternative to high-energy shock, to revert induced VFVT. Low-energy shock has a very high success rate for VT slower than VFVT. Clinical studies are required prior to consideration for empiric programming.     

Effect of telmisartan on QT interval variability and autonomic control in hypertensive patients with left ventricular hypertrophy

Objectives

The aim of the study was to examine the effect of the antihypertensive AT1 receptors antagonist telmisartan on cardiovascular autonomic function and QT dispersion in hypertensive patients with LVH.

Methods

Twenty-five patients (18 males and seven women, mean age 49.8 ± 5.2 years) with mild essential arterial hypertension and LVH were compared with 25 age-matched healthy controls. All the participants underwent a complete clinical examination, including electrocardiogram for QT interval measurements and 24 h ambulatory ECG monitoring for measurement of heart rate variability. The ECG, 24 h ambulatory ECG, and echocardiogram were repeated after eight weeks of treatment.

Results

At baseline, hypertensive patients showed QT dispersion (p < 0.001) and QTc dispersion (p < 0.001) significantly higher than control subjects. An eight-week telmisartan treatment significantly reduced blood pressure (p < 0.0001), without significant change in left ventricular mass. Telmisartan-based treatment induced an increased vagal activity without significant change of sympathetic activity and a reduction of QT dispersion (p < 0.001) and QTc dispersion (p < 0.001).

Conclusions

These data suggest that therapy with telmisartan significantly improves the sympathovagal balance increasing parasympathetic activity, and cardiac electrical stability reducing the heterogeneity of ventricular repolarization in hypertensive subjects. These effects could contribute to reduce arrhythmias as well as sudden cardiac death in at-risk hypertensive patients.     

Problems in Managing Patients with Long QT Syndrome and Implantable Cardioverter Defibrillators: A Report of Two Cases

Implantable cardioverter-defibrillators (ICDs) have been a successful adjunct to the management of arrhythmias in patients with Long QT syndrome (LQTS). In two patients, interactions between LQTS and the ICD were diagnosed and corrected. Oversensing of T waves was confirmed in the first, while in the second, the arrhythmia disappeared when T wave abnormalities improved after cessation of H₂ blocker therapy. In patients with LQTS and an ICD, T wave oversensing should be considered. Interventions that may have an adverse effect on repolarization should be avoided.     

Effect of ventricular fibrillation duration on the defibrillation threshold in humans

Early during ventricular fibrillation, the defibrillation threshold may be low, as ventricular fibrillation most probably arises from a localized area with only a few wavefronts and the effects of global ischemia, ventricular dilatation, and sympathetic discharge have not yet fully developed. The purpose of this study was to explore the effect of the timing of shock delivery in humans. During implantation of an ICD in 26 patients (24 men, 60 +/- 11 years, 19 coronary artery disease, NYHA 2.2 +/- 0.4, left ventricular ejection fraction 0.42 +/- 0.16), the defibrillation threshold was determined after approximately 10 and 2 seconds of ventricular fibrillation. Ventricular fibrillation was induced by T wave shocks. Mean defibrillation threshold was 9.9 +/- 3.6 J after 10.3 +/- 1.0 seconds. Within 2 seconds, 20 of 26 patients could be successfully defibrillated with < or = 8 J. In these patients, the mean defibrillation threshold was 4.0 +/- 2.1 J after 1.4 +/- 0.3 seconds compared to 9.5 +/- 3.1 J after 10.2 +/- 1.1 seconds (P < 0.001). There were no clinical differences between patients who could be successfully defibrillated within 2 seconds and those patients without successful defibrillation within 2 seconds. In the majority of patients, the defibrillation threshold was significantly lower within the first few cycles of ventricular fibrillation than after 10 seconds of ventricular fibrillation. These results should lead to exploration of earlier shock delivery in implantable devices. This could possibly reduce the incidence of syncope in patients with rapid ventricular tachyarrhythmias and ICDs.     

Rhythm abnormalities in children with isolated ventricular noncompaction

BACKGROUND: Isolated ventricular noncompaction (IVNC) is a serious cardiomyopathy with a generally poor prognosis. It is characterized by the presence of prominent ventricular myocardial trabeculations and deep intertrabecular recesses, in the absence of other structural heart defects. This cardiomyopathy is usually associated with ventricular dysfunction, thromboembolic events, and rhythm problems. METHODS AND RESULTS: This article describes 11 children who have rhythm abnormalities associated with IVNC. On admission, eight children had complaints including palpitation, syncope, heart failure, and bradycardia. Ventricular arrhythmias were diagnosed in four children, sinus node and atrioventricular conductance disturbances in six children, and Wolff-Parkinson-White syndrome and associated tachycardia in one child. Three children with ventricular arrhythmias received an automatic implantable cardioverter defibrillator (ICD). Three patients died of cardiac problems during the follow-up period. CONCLUSION: Since many of rhythm abnormalities, including life-threatening ventricular arrhythmias, may be seen in patients with IVNC, children with IVNC should be screened for arrhythmias. An ICD may be the best treatment for some of these patients.