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Introduction: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a cardiac inherited arrhythmogenic disease potentially leading to sudden cardiac death that is determined by electrical instability exacerbated by acute adrenergic tone., Methods and Results: Despite its life-threatening nature, CPVT remains potentially unnoticed since diagnosis may be difficult especially in apparently healthy athletes. This review summarizes current knowledge and shortcomings of CPVT, focusing on genetics, arrhythmic mechanisms, sport preparticipation screening, and current recommendations., Conclusions: The paper captures the importance of CPVT athletes regarding the necessity of risk stratification, as well as the importance of maintaining a healthy lifestyle., (© 2023 Wiley Periodicals LLC.)

Intense exercise-induced right ventricular remodeling is a potential adaptation of cardiac function and structure. The features of the remodeling may overlap with those of a very early form of arrhythmogenic right ventricular cardiomyopathy (ARVC): at this early stage, it could be difficult to discriminate ARVC, from exercise-induced cardiac adaptation that may develop in normal individuals. The purpose of this paper is to discuss which exercise-induced remodeling may be a pathological or a physiological finding. A complete evaluation may be required to identify the pathological features of ARVC that would include potential risk of sudden cardiac death during sport or, to avoid the false diagnosis of ARVC. The most recent expert assessment of arrhythmogenic cardiomyopathy focuses on endurance athletes presenting with clinical features indistinguishable from ARVC., (© 2020 Wiley Periodicals LLC.)

Background: Micra transcatheter pacemaker system (TPS) usually achieves low implant pacing threshold (IPT). However, IPT may increase in some patients during follow-up., Aim: To apply implant parameters in predicting long-term occurrence of very high pacing threshold (VHPT) in patients with Micra-TPS., Methods: A cohort of 110 consecutive patients implanted with a Micra-TPS from 2014 to 2018 was evaluated at discharge and at 1, 12, 24, 36, and 48 months follow-up. VHPT was defined as greater than 2 V/0.24 ms. VHPT predictors were identified., Results: Micra-TPS was implanted successfully in 108 patients (98.2%). During a mean follow-up of 24 ± 16 months, 18 patients (16.7%) died of causes nonpacemaker-related, and 4 (3.8%) developed VHPT. Patients with VHPT had higher IPT and lower implant impedance than patients with non-VHPT: 1 ± 0.31 vs 0.55 ± 0.29 V/0.24 ms (P = .003) and 580 ± 59 vs 837 ± 232 Ω (P = .03), respectively. IPT and impedance had excellent discriminative power to predict VHPT (area under the curve: 0.85 ± 0.07 and 0.91 ± 0.05, respectively). Negative predictive value (NPV) of IPT ≤ 0.5 V/0.24 ms was 100%; positive predictive value (PPV) was 8% throughout follow-up. Implant impedance ≤ 600 Ω had NPV of 99% throughout follow-up, whereas PPV varied: 16%, 21%, 16%, and 28% at 1, 12, 24, and 36 months, respectively. Sequential combination of IPT greater than 0.5 V/0.24 ms and impedance ≤ 600 Ω improved PPV to 25%, 35%, 27%, and 44%, respectively, whereas NPV remained 99% throughout follow-up., Conclusion: Despite favorable long-term electrical performance of Micra-TPS, a small percent of patients developed VHPT during follow-up. A sequential combination of IPT and impedance could allow the implanter to identify patients who will develop VHPT during long-term follow-up., (© 2020 Wiley Periodicals, Inc.)

Introduction: The purpose of this study was to compare the anatomical characteristics of scar formation achieved by visual-guided laser balloon (Laser) and radiofrequency (RF) pulmonary vein isolation (PVI), using late-gadolinium-enhanced cardiac magnetic resonance imaging (LGE-CMR)., Methods and Results: We included 17 patients with paroxysmal or early persistent drug resistant AF who underwent Laser ablation; 2 were excluded due to procedure-related complications. The sample was matched with a historical group of 15 patients who underwent PVI using RF. LGE-CMR sequences were acquired before and 3 months post-PVI. Ablation gaps were defined as pulmonary vein (PV) perimeter sections showing no gadolinium enhancement. The number of ablation gaps was lower in Laser versus RF ablations (median 7 vs. 14, P  =  0.015). Complete anatomical PVI (circumferential scar around PV, without gaps) was more frequently achieved with Laser than with RF (39% vs. 19% of PVs, P  =  0.025). Fewer gaps were present at the superior and anterior left PV and posterior right PV antral regions in the Laser group, compared to RF. Scar extension into the PVs was similar in both groups, although RF produced more extensive ablation scar toward the LA body. AF recurrences at 1 year were similar in both groups (Laser 36% vs. RF 27%, P  =  1.00)., Conclusions: Compared to RF, Laser ablation achieved more complete anatomical PVI, with less LA scar extension. However, AF recurrence appears to be similar after Laser compared to RF ablation. Further studies are needed to assess whether the anatomical advantages of Laser ablation translate into clinical benefit in patients with AF., (© 2018 Wiley Periodicals, Inc.)

Introduction: To investigate the relation between left atrial (LA) volume, sphericity, and fibrotic content derived from contrast-enhanced cardiac magnetic resonance imaging (CE-CMR) and their impact on the outcome of catheter ablation for atrial fibrillation (AF)., Methods and Results: In 83 patients undergoing catheter ablation for AF, CE-CMR was used to assess LA volume, sphericity, and fibrosis. There was a significant correlation between LA volume and sphericity (R  =  0.535, P < 0.001) and between LA volume and fibrosis (R  =  0.241, P  =  0.029). Multivariate analyses demonstrated that LA volume was the strongest independent predictor of AF recurrence after catheter ablation (1.019, P  =  0.018)., Conclusion: LA volume, sphericity, and fibrosis were closely related; however, LA volume was the strongest predictor of AF recurrence after catheter ablation., (© 2018 Wiley Periodicals, Inc.)

Today, understanding the true risk of adverse events in long-QT syndrome (LQTS) populations may be extremely complex and potentially dependent on many factors such as the affected gene, mutation location, degree of QTc prolongation, age, sex, and other yet unknown factors. In this context, risk stratification by genotype in LQTS patients has been extremely difficult, also during exercise practice, especially due to the lack of studies that would lead to a better understanding of the natural history of each mutation and its impact upon athletes. The creation of individualized guidelines for sport participation is a goal yet to be achieved not only due to the complexity of genotype effect on the phenotype in this patient population, but also due to penetrance in genotype-positive patients. This article summarizes current knowledge and raises questions concerning the difficult relationship between exercise practice and LQTS., (© 2018 Wiley Periodicals, Inc.)

Background: Left atrial (LA) sphericity (LASP) is a new remodeling parameter based on LA shape analysis, with independent predictive value for recurrence after atrial fibrillation (AF) ablation., Objectives: To evaluate the association between LASP and thromboembolic events (TE) in patients with AF., Methods: Twenty-nine AF patients and prior TE and 29 age- and gender-matched controls were included. LASP was calculated using a 3D-LA reconstruction. The LA appendage (LAA) volume and morphology were assessed. ROC curve analysis was performed for LASP, LA volume, LAA volume, and CHAD/CHA2 D-VASc scores (Stroke2 -the grouping variable-was excluded)., Results: Mean age of the study population was 61 ± 11 years (79.3% males, 53.4% hypertension, 8.6% diabetes). Patients with prior TE had higher LASP than those without (82.5 ± 3.3% vs. 80.2 ± 3.1%, P = 0.008); there were no differences in CHAD or CHA2 D-VASc scores, LA volume, LAA volume, or LAA morphology. The C-statistic was higher for LASP (0.71) than for other tested variables (CHAD score = 0.58, CHA2 D-VASc score = 0.59, LA volume = 0.50, LAA volume = 0.46; P < 0.01 for all vs. LASP). The best cutoff value for LASP was 83.6% (sensitivity 0.52, specificity 0.90). Logistic regression analysis showed predictive value for LASP (OR 1.26 per each 1% increase [1.85-52.20], P = 0.013), but not for clinical risk scores. The addition of LASP to the CHAD and CHA2 D-VASc scores increased the predictive value over the risk scores alone (P = 0.004), and reclassified 45.5% of patients with CHAD = 0 (no anticoagulation indicated) to moderate-risk (anticoagulation indicated)., Conclusion: LA sphericity is associated with prior TE in AF patients and improves the performance of the CHAD and CHA2 D-VASc scores alone., (© 2016 Wiley Periodicals, Inc.)

Introduction: The diagnosis of Brugada syndrome based on the ECG is hampered by the dynamic nature of its ECG manifestations. Brugada syndrome patients are only 25% likely to present a type 1 ECG. The objective of this study is to provide an ECG diagnostic criterion for Brugada syndrome patients that can be applied consistently even in the absence of a type 1 ECG., Methods and Results: We recorded 67-lead body surface potential maps from 94 Brugada syndrome patients and 82 controls (including right bundle branch block patients and healthy individuals). The spatial propagation direction during the last r' wave and the slope at the end of the QRS complex were measured and compared between patients groups. Receiver-operating characteristic curves were constructed for half of the database to identify optimal cutoff values; sensitivity and specificity for these cutoff values were measured in the other half of the database. A spontaneous type 1 ECG was present in only 30% of BrS patients. An orientation in the sagittal plane < 101º during the last r' wave and a descending slope < 9.65 mV/s enables the diagnosis of the syndrome with a sensitivity of 69% and a specificity of 97% in non-type 1 Brugada syndrome patients., Conclusion: Spatiotemporal characteristics of surface ECG recordings can enable a robust identification of BrS even without the presence of a type 1 ECG., (© 2016 Wiley Periodicals, Inc.)

Background: Atrial fibrillation (AF) is the most common sustained cardiac arrhythmia imposing substantial morbidity and mortality. Catheter-based pulmonary vein isolation (PVI) using radiofrequency current (RFC) has become a standard treatment for drug-resistant and symptomatic paroxysmal AF (PAF). In recent years, the cryoballoon-based technique is increasingly used as a promising alternative with a short learning curve., Methods: The FIRE AND ICE trial is a prospective, randomized, controlled, open, blinded outcome assessment, noninferiority trial comparing cryoballoon-, and RFC-based PVI. Patients with drug-resistant PAF will be randomized in a 1:1 matrix in multiple European centers. The primary hypothesis is that cryoballoon ablation is not inferior to RFC ablation using 3-dimensional mapping with respect to clinical efficacy. The primary endpoint is defined as the time to first documented clinical failure, including: (1) recurrence of AF; (2) atrial flutter or atrial tachycardia; (3) prescription of class I or III antiarrhythmic drugs; or (4) re-ablation, whichever comes first, following a blanking period of 3 months after the index ablation procedure. The primary safety endpoint is a composite of death, stroke/transient ischemic attack, cardiac arrhythmias (apart from AF recurrence) causally related to the therapeutic intervention, and procedure-related serious adverse events., Conclusion: The FIRE AND ICE trial compares 2 different technologies to perform catheter ablation of PAF with respect to efficacy and safety. It aims at providing objective data to guide selection and usage of ablation catheters in the treatment of AF., (© 2014 Wiley Periodicals, Inc.)

Background: Optimization of atrioventricular (AV) and interventricular (VV) intervals may improve cardiac resynchronization therapy (CRT) response but is a complex task. Fusion with intrinsic conduction may increase the benefit of CRT. The aim was to describe fusion-optimized intervals (FOI), a new method of optimizing CRT based on QRS duration., Methods and Results: Seventy-six consecutive patients with preserved AV conduction who received CRT were prospectively included. The AV interval was optimized by searching the narrowest QRS obtained within the fusion band during left ventricular (LV) pacing. The VV interval was then adjusted, comparing QRS duration in simultaneous biventricular, LV preexcitation (-30 milliseconds), right ventricular (RV) preexcitation (-30 milliseconds) and LV-only pacing. A substudy in 31 patients evaluated the invasive LV +dP/dtmax . The best fusion-optimized AV interval was 136 ± 30 milliseconds during atrial sensing and 192 ± 35 milliseconds during atrial pacing. The best QRS was obtained with simultaneous biventricular pacing in 28 patients (37%), LV preexcitation in 22 (29%), LV-only in 20 (26%), and RV preexcitation in 6 (8%). Baseline QRS was shortened more by FOI (59 ± 19 milliseconds) than by nominal settings (40 ± 21 milliseconds; P < 0.001). Sixty-five patients (86%) showed >10% shortening of the baseline QRS with FOI; none prolonged the QRS duration by FOI compared to nominal settings. All echocardiographic asynchrony parameters were corrected by FOI. Baseline +dP/dtmax improvement was greater in FOI (127 ± 95 mmHg/seconds) than in nominal settings (102 ± 71 mmHg/seconds; P = 0.05)., Conclusion: The FOI method is feasible, further reduces QRS duration, and improves acute hemodynamic response compared to nominal programming of CRT., (© 2013 Wiley Periodicals, Inc.)

Background: Atrial fibrillation (AF) ablation outcome is mainly determined by atrial remodeling that, nowadays, is only estimated through clinical presentation (persistent vs. paroxysmal) and left atrial (LA) dimension. The aim of the study was to stage the atrial remodeling process using the Left Atrial Sphericity (LASP) and determine whether this technique may help to predict AF ablation outcome., Methods: Consecutive patients who underwent contrast-enhanced cardiac magnetic resonance angiography before AF ablation were included in the study. Three-dimensional reconstruction of LA excluding pulmonary veins and the LA appendage was used to define the LA cavity. The LASP was automatically obtained with self-customized software., Results: 106 patients were included and categorized in 3 groups (Gs): discoid-LA (G1), intermediate-LA (G2), and spherical-LA (G3). The G3 patients had larger LA anteroposterior diameter than G1 and G2 patients (47 ± 7 vs 43 ± 6 and 39 ± 5 mm; P < 0.001), greater LA volume (90 ± 39 vs 86 ± 24 and 73 ± 20 mm; P = 0.012), and higher prevalence of persistent AF (75% vs 48% and 29%; P = 0.034) structural heart disease (75% vs 19% and 19%; P < 0.001), and AF recurrence at 12 months follow-up (58% vs 29% and 5%, P < 0.001). The LASP had linear correlation to predicted probability of recurrence. Multivariate analysis identified LASP (OR 1.320 [1.096-1.591], P = 0.004) and hypertension (OR 3.694 [1.282-10.645]; P = 0.016) as independent risk factors for arrhythmia recurrence., Conclusion: Left Atrial Sphericity is a new independent predictor of recurrence after AF ablation and may be useful in selecting the best candidates for AF ablation., (© 2013 Wiley Periodicals, Inc.)

Introduction: Echocardiographic optimization of the VV interval may improve CRT response, but it is time-consuming and not routinely performed. The aim of this study was to compare the response to cardiac resynchronization therapy (CRT) when the interventricular pacing (VV) interval was optimized by tissue Doppler imaging (TDI) to CRT response when it was optimized following QRS width criteria., Methods and Results: The study included 156 consecutive CRT patients with severe heart failure and left bundle-branch block configuration. Atrioventricular interval was selected according to a pulsed Doppler assessment, and VV optimization was randomly assigned to echocardiography (ECHO group, n = 78) or electrocardiography (ECG group, n = 78). Optimal VV was defined for the ECHO group as producing the best LV intraventricular synchrony according to TDI displacement curves and for the ECG group as resulting in the narrowest QRS measured from the earliest deflection. At 6-month follow-up, percentage of echocardiographic responders (defined as neither death nor heart transplantation and a LV end-systolic volume reduction >10%) was higher in the ECG optimized group (50.0% vs 67.9%; P = 0.023), whereas clinical response (defined as neither death nor heart transplantation and >10% improvement in the 6-minute walking test) was similar in both groups (71.8% vs 73.1%; P = 0.858)., Conclusions: VV optimization based on QRS width obtained a higher percentage of responders in terms of LV reverse remodeling compared to the TDI method., (© 2011 Wiley Periodicals, Inc.)

Brugada Syndrome and Ketamine Overdose.  A 31-year-old man was referred for ketamine overdose. He presented initially with transient major Brugada ECG pattern. Complete investigation led to the diagnosis of slowly resolvent toxic myocarditis. Brugada-like ECG was suspected to be caused by the ketamine intoxication in this case., (© 2010 Wiley Periodicals, Inc.)

Introduction: The Atrial Fibrillation (AF) Exchange Group, an international multidisciplinary group concerned with the management of AF, was convened to review recent advances in the field and the potential impact on treatment strategies., Methods: Issues discussed included epidemiology and the impact of the rising incidence of AF on health care systems, developments in pharmacological and surgical interventions in the management of arrhythmias and thromboprophylaxis, the potential to affect treatment strategies, and barriers to implementing them., Results: The incidence of AF and the associated burden on health care systems are increasing with aging populations, prevalence of comorbidities and more effective treatment of cardiovascular diseases. Advances in available medical treatments, in particular dronedarone and dabigatran, with other products in development, offer the possibility of changes in treatment paradigms and a greater emphasis on reducing hospitalizations and improvement in long-term outcomes instead of a symptom/safety-driven approach in which the priority is symptom suppression without provoking drug toxicity. Developments in catheter ablation techniques may mean that, in experienced centers, ablation may be offered as first-line treatment in selected patient populations. Barriers to optimal treatment include underdiagnosis, lack of recognition as a serious condition and as a risk factor for stroke, limited access to care, inadequate implementation of guidelines, and poor adherence to treatment., Conclusions: The focus of the management of AF may be changing as a consequence of new treatments based on the outcome improvements they offer. However, the benefits will not be fully realized if guidelines and guidance are not observed in routine clinical practice.

Introduction: Measurement of left ventricular (LV) asynchrony is usually determined on single time points from spectral tissue Doppler imaging (TDI) scans that are frequently difficult to identify or not representative of the whole cardiac cycle. Our aim was to validate a new asynchrony index that evaluates the motion of the LV walls throughout the whole cardiac cycle., Methods and Results: Ten healthy volunteers and 50 patients undergoing cardiac resynchronization therapy (CRT) were studied with TDI. Wall displacement tracings from the septal and lateral LV walls were analyzed. Cross-correlation was calculated and 2 indices were obtained to assess LV asynchrony: the time delay and the superposition index (SI) between wall displacements. These results were compared between healthy volunteers and CRT patients, and between responders and nonresponders to CRT. Also, the optimal interventricular (VV) interval was based upon the best matching level. Volunteers showed lower asynchrony indices (83 +/- 2% SI, 17 +/- 8 ms time delay) as compared with CRT patients (63 +/- 15% SI, 73 +/- 60 ms time delay, P < 0.05). Responders also had more LV dyssynchrony than nonresponders (58 +/- 15% SI and 92 +/- 66 ms vs 68 +/- 12% and 48 +/- 34 ms, P < 0.05). The optimum VV interval selected by the computed algorithm showed an excellent concordance (Kappa = 0.90, P < 0.05) with that determined by other validated methods for optimizing the programming of CRT devices., Conclusions: This approach allows measurement of LV intraventricular asynchrony throughout the cardiac cycle, being useful to determine the optimum VV interval and to select candidates for CRT.

Background: Brugada syndrome predisposes some subjects to ventricular tachyarrhythmias and sudden cardiac death. Mutations in SCN5A gene have been associated with approximately 25% of Brugada syndrome patients. A common variant in SCN5A, H558R has shown to improve sodium channel activity in mutated channels. We studied whether common variant H558R has any clinical implications in the phenotype of Brugada syndrome., Methods: Our study population consisted of Brugada syndrome subjects 75 with SCN5A mutation and 92 without SCN5A mutation. Their mean age was 39 +/- 15 and 42 +/- 17 years, and 65% and 86% were male, respectively. We measured PR-, QRS-, QTc-intervals from leads II and V2 of the 12-lead ECG. We also evaluated J-point amplitude from lead V2 and R'/S ratio from lead aVR (the "aVR sign"). The H558R (A-->G) genotype was detected with direct sequencing of the SCN5A gene., Results: The AA genotype carriers had longer QRS duration in lead II (P = 0.017) and higher J-point elevation in lead V2 (P = 0.013), higher "aVR sign" (P = 0.005) and a trend toward more subjects with symptoms (P = 0.067) than G allele carriers. None of the results were significant in Brugada syndrome subjects without SCN5A mutation., Conclusion: The common variant H558R seems to be a genetic modulator of Brugada syndrome among carriers of a SCN5A mutation, in whom the presence of the less common allele G improves the ECG characteristics and clinical phenotype.

Introduction: The spontaneous presence of a coved-type ECG is considered as an important risk factor in Brugada syndrome. However, diagnosis making and risk stratification may be hampered by the dynamic nature of the ECG abnormalities. The objective of this study was to determine the variability and predictive value of the electrocardiogram in Brugada patients implanted with a cardioverter-defibrillator (ICD)., Methods and Results: We analyzed consecutive 12-lead ECGs from 89 ICD patients (44 +/- 14 years, 69 males) with Brugada syndrome. A total of 1,161 ECGs were included for analysis (13 +/- 8 ECGs/patient). Twenty-four percent of the ECGs/patient were coved-type I, 25% saddleback-type II or III, and 51% normal. Fifty-seven patients had a diagnostic coved-type ECG spontaneously (group A), 32 patients only after drug challenge (group B). In group A, 38% of the ECGs/patient were diagnostic, 25% saddleback-type, and 37% normal. Fifty-five group A patients (96%) had transient normalization and/or conversion to saddleback-type ECGs. During a mean follow-up of 48 +/- 35 months, 16 patients (18%) experienced appropriate shocks. All patients with appropriate shocks had spontaneous diagnostic ECGs. They tended to have more coved-type ECGs (36 vs 22%, respectively, P = 0.05) than patients without appropriate shocks., Conclusions: Analysis of serial ECG recordings in an ICD patient population shows that the Brugada-ECG pattern is highly variable over time. In patients with spontaneous coved-type ECG, only every third ECG is diagnostic and every third ECG normal. Patients with spontaneous coved-type ST-segment elevation have a high incidence of appropriate shocks. Spontaneous saddleback-type electrocardiograms are not helpful for risk stratification.

Introduction: Brugada syndrome (BrS) is an inherited disorder that predisposes some subjects to sudden cardiac death (SCD). It is not well established which BrS patients are at risk of severe arrhythmias. Our aim was to study whether standard 12-lead electrocardiogram (ECG) would give useful information for this purpose., Methods: This study included 200 BrS probands (142 male, 62%; mean age 42 +/- 16 years). Symptoms related to BrS were defined as syncope, documented ventricular tachyarrhythmia, or SCD. We determined PR, QRS, QTc, T(peak), and T(end) interval from leads II and V(2) and QRS from lead V(5), R'/S ratio from lead aVR (aVR sign), QRS axis, and J-point elevation amplitude from right precordial leads from the baseline ECGs., Results: Sixty-six subjects (33%) had experienced symptoms related to BrS. The only significant difference between the symptomatic and asymptomatic BrS subjects was the QRS duration measured from lead II or lead V(2), for example, the mean QRS in V(2) was 115 +/- 26 ms in symptomatic versus 104 +/- 19 ms in asymptomatic patients (P < 0.001). The optimized cut-off point of V(2) QRS > or =120 ms gave an odds ratio (OR) of 2.5 (95% CI: 1.4-4.6, P = 0.003) for being symptomatic. In a multivariate analysis adjusted with gender, age, and SCN5A mutation, the OR was 2.6 (95% CI: 1.4-4.8, P = 0.004)., Conclusion: Prolonged QRS duration, measured from standard 12-lead ECG, is associated with symptoms and could serve as a simple noninvasive risk marker of vulnerability to life-threatening ventricular arrhythmias in BrS.

Introduction: Circumferential pulmonary vein ablation (CPVA) for atrial fibrillation (AF) consists of creating extensive lesions in the left atrium (LA). The aim of the study was to evaluate changes in LA contractility after ablation and their relationship with procedure outcome., Methods and Results: A series of 90 consecutive patients underwent cardiac magnetic resonance imaging (MRI) before and 4-6 months after CPVA. Only patients in sinus rhythm during both imaging acquisitions were included in the study to measure LA end-diastolic (LAmax) and LA end-systolic (LAmin) volumes. Fifty-five patients were finally analyzed (41 men, 52 +/- 11 years, 74% paroxysmal AF). During a mean follow-up of 12 +/- 7 months and after 1.2 +/- 0.3 ablation procedures, 38 patients (69%) were arrhythmia-free (group I), and the remaining 17 patients had recurrences (group II). There was a significant decrease in mean LAmax volume in both groups, whereas mean LAmin volume only decreased in group I. Mean LA ejection fraction (EF) was preserved after CPVA in group I (40 +/- 11% vs 38 +/- 10%; P = 0.27) but decreased in patients with arrhythmia recurrences (37 +/- 10% vs 27 +/- 10%; P < 0.001). In fact, LA EF remained stable or increased in 68% of patients without arrhythmia recurrences., Conclusions: LAmax volume reduction following CPVA occurs regardless of the clinical efficacy of the procedure, whereas mean LAmin volume only decreased in patients without recurrences. LA EF was preserved or even increased in most patients with successful CPVA.

Introduction: Echocardiography is widely used to optimize CRT programming, but it is time-consuming. This study aimed to correlate the optimal interventricular pacing (V-V) interval obtained by echo with the optimal V-V interval obtained by a simpler method based on the surface ECG., Methods and Results: Three V-V intervals were tested: LV preactivation at -30 ms, simultaneous biventricular pacing (0 ms), and RV preactivation at +30 ms. The one that achieved the best LV synchrony was chosen as the optimal V-V. This result was then compared with two different ECG measurements. The first ECG method considered the best V-V to be that which achieved the narrowest QRS. The second V-V method consisted in measuring the interval from the pacing spike to the beginning of the fast deflexion of the QRS complex in leads V1, V2, first pacing from the LV (T1), and after from the RV (T2). The T2-T1 interval was considered as a surrogate measurement of interventricular delay and defined as the best V-V. A cohort of 31 consecutive patients treated with CRT was studied. Optimal V-V interval obtained by echo was -30 ms in 25 patients (80%), +30 ms in three patients (10%), and 0 ms in the remaining three patients (10%). Echo results had 32% coincidence with the first ECG method (r = 0.2, P = NS) and 83% coincidence with the second ECG method (r = 0.81 P< 0.001)., Conclusions: The time difference in the fast ventricular depolarization observed between RV and LV stimulation in the surface ECG shows a good correlation with the V-V optimization chosen according to echo.

Introduction: PRKAG2 plays a role in regulating metabolic pathways, and mutations in this gene are associated with familial ventricular preexcitation, hypertrophic cardiomyopathy, and atrioventricular conduction disturbances. Clinico-pathologic and experimental data suggest the hypothesis of a glycogen storage disease., Objective: To report a unique pattern of clinical features observed in individuals with a mutant PRKAG2 from two unrelated families., Methods and Results: We studied two large families and found a total of 20 affected individuals showing a combination of sinus bradycardia, short PR interval, RBBB, intra and infrahisian conduction disturbances often requiring a pacemaker, and atrial tachyarrhythmias. Three individuals died suddenly at a young age. No patient had the Wolff-Parkinson-White (WPW) syndrome, and only two patients (10%) had myocardial hypertrophy. We performed screening of the exons and exon-intron boundaries of PRKAG2. Genetic analysis revealed a missense mutation (Arg302Gln) in the affected individuals from both families. This mutation had been described before and has been associated with the familial form of the WPW syndrome and with a high prevalence of left ventricular hypertrophy., Conclusion: PRKAG2 mutations are responsible for a diverse phenotype and not only the familial form of the WPW syndrome. Familial occurrence of right bundle branch block, sinus bradycardia, and short PR interval should raise suspicion of a mutant PRKAG2 gene.

Pacemaker-mediated tachycardia is a well-known complication of dual-chamber devices. In this report, we describe for the first time a case of orthodromic pacemaker-mediated tachycardia in a patient in whom a biventricular system without an atrial electrode had been implanted. Retrograde atrial activation was directly produced by the dislodged coronary vein electrode in the AV groove, resulting in simultaneous capture of the left atrium and left ventricle. During tachycardia, AV nodal conduction was via the anterograde pathway of the circuit and limited the ventricular response. Subsequently, right ventricular activation was sensed by the right ventricular electrode that triggered biventricular pacing and left atrial capture, perpetuating the tachycardia. Because the left atrial threshold was higher than the left ventricular threshold, the problem could be resolved easily by lowering the output of the coronary vein electrode.

Introduction: Brugada syndrome is characterized by sudden death secondary to malignant arrhythmias and the presence of ST segment elevation in leads V(1) to V(3) of patients with structurally normal hearts. This ECG pattern often is concealed but can be unmasked using potent sodium channel blockers. Like congenital long QT syndrome type 3 (LQT3) and sudden unexpected death syndrome, Brugada syndrome has been linked to mutations in SCN5A., Methods and Results: We screened a large European family with Brugada syndrome. Three members (two female) had suffered malignant ventricular arrhythmias. Ten members showed an ECG pattern characteristic of Brugada syndrome at baseline, and eight showed the pattern only after administration of ajmaline (total 12 female). Haplotype analysis revealed that all individuals with positive ECG at baseline shared the SCN5A locus. Sequencing of SCN5A identified a missense mutation, R367H, previously associated with sudden unexpected death syndrome. Two of the eight individuals who displayed a positive ECG after the administration of ajmaline, but not before, did not have the R367H mutation, and sequencing analysis failed to identify any other mutation in SCN5A. The R367H mutation failed to generate any current when heterologously expressed in HEK cells., Conclusion: Our results support the hypothesis that (1) sudden unexpected death syndrome and Brugada syndrome are the same disease; (2) male predominance of the phenotype observed in sudden unexpected death syndrome does not apply to this family, suggesting that factors other than the specific mutation determine the gender distinction; and (3) ajmaline may provide false-positive results. These findings have broad implications relative to the diagnosis and risk stratification of family members of patients with the Brugada syndrome.

Introduction: The prognostic value of electrophysiologic studies in individuals with the syndrome of right bundle branch block and ST segment elevation in precordial leads V1 to V3 (Brugada syndrome) remains controversial. Our previous data from 252 individuals with the syndrome suggested that programmed ventricular stimulation had a good overall accuracy to predict events. However, studies from independent investigators questioned our results. We report here the largest population with Brugada syndrome ever studied by programmed electrical stimulation of the heart., Methods and Results: Four hundred forty-three individuals with an ECG diagnostic of Brugada syndrome were studied by programmed electrical stimulation of the heart. The diagnosis was made because of the classic ECG showing a coved-type ST segment elevation in precordial leads V1 to V3. Of the 443 individuals, 180 had developed spontaneous symptoms (syncope or aborted sudden cardiac death) and 263 were asymptomatic at the time the diagnosis was made. The ventricular stimulation protocol included a minimum of two basic pacing cycle lengths with two ventricular premature beats from the right ventricular apex. A sustained ventricular arrhythmia was induced in 217 cases (49%). Symptomatic patients were more frequently inducible [126/180 (70%)] than asymptomatic individuals [91/263 (34%); P = 0.0001]. Males were more frequently inducible than females (54% vs 32%, P < 0.0001). Inducible individuals had a longer HV interval than noninducible patients (50 +/- 12 msec vs 46 +/- 10 msec, P < 0.002). HV interval and number of premature beats needed to induce VF were not related to outcome. Inducibility was statistically a powerful predictor of arrhythmic events during follow-up. Sixty of 217 inducible patients (28%) had spontaneous ventricular fibrillation compared with 5 of 221 noninducible patients (2%; P = 0.0001)., Conclusion: Inducibility of sustained ventricular arrhythmias during programmed ventricular stimulation of the heart is a good predictor of outcome in Brugada syndrome.

Introductions: Location of the accessory pathway (AP) in Wolff-Parkinson-White (WPW) syndrome can be determined accurately by the QRS polarity on resting ECG. These ECG characteristics may be different in children, and no algorithm has yet been tested., Methods and Results: A total of 153 resting ECGs of symptomatic children with WPW syndrome were retrospectively analyzed. The anatomic AP location had been established fluoroscopically at eight possible sites during radiofrequency catheter ablation. Two independent observers predicted AP location on blinded ECGs with a QRS polarity algorithm for adults using leads II, III, aVL, V1, and V2. Subsequently, the QRS polarity for all individual ECG leads was evaluated and a new algorithm for children was devised. With the adult algorithm, the observers correctly predicted only 55% to 58% of AP locations. The septal and right-sided pathways often were inseparable, and mid-septal and parahisian pathways were missed. In the new children's algorithm, left lateral, left posteroseptal, and posteroseptal pathways shared a positive or intermediate QRS polarity on V1, with the left lateral pathway separated by a positive QRS polarity on lead III. Negative QRS polarity on lead V1 and positive QRS polarity on lead V3 were shared by right posteroseptal, mid-septal, parahisian, and anteroseptal pathways, with the latter two having a positive QRS polarity on lead aVF. Right lateral pathways had negative QRS polarity on lead V1 and negative or intermediate QRS polarity on lead V3. Overall accuracy for these five regions was 90%., Conclusion: AP characterization by QRS polarity in children with WPW syndrome is more diverse than in adults and requires other ECG leads to establish five AP regions.

Use of different classifications for atrial fibrillation reflects the complexity of the arrhythmia and the difficulty in grouping its different aspects. Current classifications are based on clinical presentation, etiology, substrates, mechanisms, etc. From the clinical point of view, the most relevant probably should be one directed at classifying patients in terms of therapeutic options. In this article, a review of known classifications is given, together with an attempt at a new classification based on the possibility of offering a nonpharmacologic treatment to patients.

Introduction: The prognostic value of electrophysiologic investigations in individuals with Brugada syndrome is unclear. Previous studies failed to determine its value because of a limited number of patients or lack of events during follow-up. We present data on the prognostic value of electrophysiologic studies in the largest cohort ever collected of patients with Brugada syndrome., Methods and Results: Two hundred fifty-two individuals with an ECG diagnostic of Brugada syndrome were studied electrophysiologically. The diagnosis was made because of a classic ECG with a coved-type ST segment elevation in precordial leads V1 to V3. Of the 252 individuals, 116 had previously developed spontaneous symptoms (syncope or aborted sudden cardiac death) and 136 were asymptomatic at the time of diagnosis. A sustained ventricular arrhythmia was induced in 130 patients (51%). Symptomatic patients were more frequently inducible (73%) than asymptomatic individuals (33%) (P = 0.0001). Fifty-two individuals (21%) developed an arrhythmic event during a mean follow-up of 34 +/- 40 months. Inducibility was a powerful predictor of arrhythmic events during follow-up both in symptomatic and asymptomatic individuals. Overall accuracy of programmed ventricular stimulation to predict outcome was 67%. Overall accuracy in asymptomatic individuals was 70.5%, with a 99% negative predictive value. Overall accuracy in symptomatic patients was 62%, with only a 4.5% false-negative rate. No significant differences were found in the duration of the H-V interval during sinus rhythm between symptomatic or asymptomatic individuals. However, the H-V interval was significantly longer in the asymptomatic individuals who became symptomatic during follow-up compared with those who did not develop symptoms (59 +/- 8 msec vs 48 +/- 11 msec, respectively; P = 0.04)., Conclusion: Inducibility of sustained ventricular arrhythmias is a good predictor of outcome in Brugada syndrome. In asymptomatic individuals, a prolonged H-V interval during sinus rhythm is associated with a higher risk of developing arrhythmic events during follow-up. Symptomatic patients require protective treatment even when they are not inducible. Asymptomatic patients can be reassured if they are noninducible.

Introduction: Enhanced detection criteria in third-generation implantable defibrillators have been implemented to avoid inappropriate therapy of fast supraventricular arrhythmias. We prospectively analyzed the use of these criteria in patients with an implantable defibrillator with electrogram storing capability., Methods and Results: In 82 consecutive patients with a Guidant-CPI implantable defibrillator, sudden onset > 9% and stability < 40 msec were systematically programmed in zone 1 of therapy together with a sustained rate duration security mechanism. All detected tachycardia episodes were analyzed. The study population consisted of 59 patients who had at least one episode of tachycardia detected in zone 1 during follow-up. The tachycardia rate in zone 1 never exceeded 210 beats/min. Twenty patients had no episodes during follow-up, and three patients had episodes detected exclusively in zone 2 of therapy. Supraventricular arrhythmias were detected frequently in the ventricular tachycardia zone (193 of 690 tachycardia episodes in 23 of 59 patients). Use of sudden onset was very effective in detecting sinus tachycardias (65 of 67 episodes), and stability was very useful in detecting atrial fibrillation (31 of 32 episodes). However, sensitivity in detecting ventricular tachycardia was only 90% (451 of 497 episodes). Application of the sustained rate duration criterion allowed appropriate treatment of all ventricular tachycardia episodes, increasing sensitivity to 100%; however, specificity in appropriate nontreatment of supraventricular decreased from 96% to 83%. Subsequent analysis of different algorithms applied to our data showed that sudden onset > 9% and stability < 40 msec was the algorithm with the best specificity and sensitivity., Conclusion: Programming sudden onset and stability detection criteria with a sustained rate duration safety net for triggering tachycardia therapy results in appropriate device management in most patients with supraventricular and slow (< 210 beats/min) ventricular tachycardias.

Introduction: Feasibility of radiofrequency (RF) ablation using a two-catheter technique without coronary sinus catheterization was studied in 100 consecutive patients with a single concealed left free-wall accessory pathway., Methods and Results: Tachycardia was induced by electrical stimulation in the right atrium/right ventricle, and the presence of a concealed left free-wall accessory pathway was suggested electrocardiographically (negative P wave in leads I and/or aVL during orthodromic tachycardia) or by earlier atrial activation in the pulmonary artery compared to the high right atrium. Mapping of the mitral annulus was performed during right ventricular pacing or orthodromic tachycardia, and RF energy was applied at the site with the earliest retrograde atrial activation. Ablation was considered effective if tachycardia could not be induced, and if VA dissociation or exclusive retrograde nodal conduction was observed. Ablation was initially successful in 98 of 100 patients. Mean number of radiofrequency pulses were 3.2 +/- 2. Mean fluoroscopy time and total procedure time was 14 +/- 9 and 107 +/- 32 minutes, respectively. There were no complications related to the procedure. At a mean follow-up of 22 +/- 13 months, two patients experienced tachycardia recurrence and required a second procedure, which was successful., Conclusions: Our results suggest that RF catheter ablation of concealed left free-wall accessory pathways can be safely, effectively, and rapidly performed using a simplified two-catheter technique with no need for coronary sinus catheterization.

We recently described a syndrome characterized by an ECG pattern of right bundle branch block and persistent ST segment elevation in leads V1 to V3 in patients suffering from aborted sudden cardiac death and not having demonstrable structural heart disease. We present new observations on this syndrome, especially those related to asymptomatic and intermittent forms. Forty-seven patients with the described ECG pattern were identified; 32 were symptomatic with syncope and sudden death aborted by cardiopulmonary resuscitation. Eleven patients received pharmacologic therapy, mainly amiodarone and/or beta-blocking agents, and 21 patients received an implantable defibrillator with or without pharmacologic therapy. Three of the 11 patients on pharmacologic therapy died suddenly during follow-up, while 9 of 21 patients with an implantable defibrillator used the device during follow-up. The remaining 15 patients were asymptomatic when first seen. Three patients died suddenly after 6 years, 3 months, and 2 months of follow-up without treatment. Another patient received an implantable defibrillator after syncope and had subsequent episodes of ventricular fibrillation terminated by the defibrillator. The other 11 patients remain asymptomatic without (6) or with (5) treatment with beta blockers. In 14 of the 47 patients, the ECG normalized momentarily during follow-up but later became abnormal again. During transient normalization of the ECG, administration of ajmaline or procainamide unmasked the described ECG pattern in six patients who received the drug. Long-term follow-up of survivors failed to show progression to any form of right or left ventricular cardiomyopathy.