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A case of permanent junctional reciprocating tachycardia with post-mortem documentation of an accessory atrioventricutar pathway as the substrate of the arrhythmia is reported. Tachycardia had lasted for 15 years and showed a retrograde P wave (P′) and R–P′ longer than P′–R interval. The tachycardia circuit utilized a concealed posterior septal accessory pathway as the retrograde limb. Because the arrhythmia was disabling and unresponsive to pharmacological treatment, the patient underwent closed chest ablation of the His bundle. After the procedure, no anterograde or retrograde conduction over the normal conduction system was observed; anterograde conduction over the anomalous pathway showed decremental properties. Because of previous myocardial infarction, the patient developed a ventricular aneurysm and died suddenly 5 months after His bundle ablation. Histological examination of the heart revealed a group of tiny fibromuscular bundles joining the lower rim of the coronary sinus outlet to the summit of the interventricular septums; the anomalous atrioventricular connection pursued a sinuous, tortuous path. The geometrical disposition of the accessory pathway may have been responsible for the decremental properties of conduction observed during life. [ABSTRACT FROM PUBLISHER]

Background: Criteria such as electrograms voltage or late potentials have been largely utilized in the past to help identify areas of substrate maps that are within the ventricular tachycardia (VT) isthmus; yet their specificity and positive predictive value are quite low. The Lumipoint fractionation tool of the Rhythmia system illuminates regions with fractionated electrograms irrespective of their timing and annotation. We aimed to ascertain whether the use of this tool can rapidly identify areas within VT isthmuses from substrate maps., Methods: Thirty patients with structural cardiomyopathy in whom a complete right ventricular-paced substrate map and a full reconstruction of the diastolic isthmus during VT could be obtained were enrolled. The VT isthmus border was projected on each substrate map to verify whether the areas illuminated by Lumipoint fell within those borders. The behavior of the electrograms detected at the illuminated areas of the substrate maps was studied during a right ventricular drive train and extra stimulus protocol: if the near field potentials showed a delayed conduction after a single extra stimulus, defined as a minimum of 10 ms increase of the time interval between the far field and the near field activation measured during the drive train, the electrogram was said to have a "decremental" behavior., Results: The logistic analysis showed that areas with fractionated electrograms illuminated by the Lumipoint software and showing the greatest decremental behavior fell within the VT isthmus borders (OR = 1.66, CI: 1.41-1.75, p<0.001; OR=1.57 CI: 1.32-1.72, p<0.001, respectively) with a sensitivity, specificity, and positive predictive value of 87%, 96%, and 97%, respectively., Conclusions: Fractionated electrograms illuminated by the automated Lumipoint software on right ventricular-paced substrate maps showing the greatest decremental behavior fall within the VT isthmus borders with a probability of 0.97, irrespective of their timing, annotation, or voltage, without any need for subjective assessment of their involvement in slow conduction areas., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background: The very high-power short-duration (vHPSD) temperature-controlled ablation (vHPSD) improves the efficiency of pulmonary vein isolation (PVI) procedures. We evaluated the procedural and 12-months outcomes in atrial fibrillation (AF) patients undergoing PVI by means of vHPSD ablation. In patients with AF or atrial tachyarrythmia (AT) recurrence undergoing a redo procedure the durability of the PVI was investigated., Methods: Consecutive paroxysmal/persistent AF patients undergoing PVI with the vHPSD ablation strategy (90 W, for 4 s) were enrolled. The rate of PVI, first-pass isolation, acute reconnection, and procedural complications were evaluated. Follow-up examinations and EKG were scheduled at 3,6, and 12 months. In case of AF/AT recurrence, patients underwent a redo procedure., Results: Overall, 163 AF patients (29 persistent and 134 paroxysmal) were enrolled. The PVI was reached in 100% of patients (88% at the first pass). The rate of acute reconnection was 2%. The radiofrequency, fluoroscopy and procedural times were respectively 5.5 ± 1 min, 9 ± 1 min and 75 ± 20 min. No death, tamponade nor steam pops occurred; however, 5 patients had vascular complications. The 12-months freedom from AF/AT recurrence was 86% in both paroxysmal and persistent patients. Overall, 9 patients underwent a redo procedure, and in 4 all veins were still isolated, whereas in 5 pulmonary vein reconnections were found. The PVI durability was 78%. No overt clinical complications were observed in the follow-up., Conclusions: The vHPSD ablation represents an effective and safe ablation strategy to achieve PVI. The 12-months follow-up showed high freedom from AF/AT recurrence and a good safety profile., (© 2023. The Author(s).)

Background: The aim of this study was to evaluate whether the benefit of cardiac resynchronization therapy with an implantable defibrillator (CRT-D) may differ among classes of indications to device therapy., Methods: All-cause mortality, first hospitalization for non-fatal heart failure, stable improvement of NYHA functional class (responders), and implant-related complications were evaluated retrospectively in 103 patients selected among those (n = 133) who received consecutively CRT-D between 2006 and 2009. Patients were divided into three groups: group IA (n = 65) included patients receiving CRT-D for a class IA indication; group IIa (n = 26) included patients with atrial fibrillation and QRS ≥ 130 ms receiving CRT-D for a class IA indication; nonconventional group (NC) (n = 12) included patients with an indication to defibrillator implantation extended to CRT-D because of NYHA class III-IV and echocardiographic evidence of electromechanical dyssynchrony. Echocardiographic examination was performed in all patients to identify wall target for left-side lead placement., Results: Group IIa patients were slightly older than group IA patients (p<0.05); gender distribution, left ventricular ejection fraction at implantation, ischemic etiology, and heart failure treatment were comparable among groups (all p>0.5), except for a higher digitalis use in group IIa patients (p<0.05). In a mean observation period of 3 years (up to December 2010), the rates of fatal events (IA: 22%, IIa: 23%, NC: 20%), rehospitalization for worsening heart failure (IA: 30%, IIa: 33%, NC: 22%), clinical responders (IA: 78%, IIa: 78%, NC: 78%), implant-related complications requiring reintervention (IA: 15%, IIa: 19%, NC: 25%), including pocket or catheter infections (IA: 5%, IIa: 11%, NC: 8%) were comparable among groups (all p>0.5)., Conclusions: In the "real world", the benefit of CRT-D in advanced heart failure patients might be comparable among class IA, IIa or NC indication.

Background: Estimating contractility of the left ventricle with noninvasive techniques is an important yet elusive goal. Positive inotropic interventions are mirrored by smaller end-systolic volumes and higher end-systolic pressures. An increased heart rate progressively increases the force of ventricular contraction (Bowditch treppe or staircase phenomenon)., Aim: To assess the feasibility of a noninvasive estimation of force-frequency relation (FFR) during pacing stress in the echo lab in patients with permanent pacemaker (PM)., Methods: Transthoracic stress pacing echocardiography was performed in 26 patients with a permanent pacemaker (age 69+/-11 years; 21 men, 5 women). Seven patients had normal function at baseline and during stress ("normals"); eight had angiographically assessed coronary artery disease (three with and five without induced ischemia with stress echo); eleven patients had dilated cardiomyopathy (DC). To build the FFR, the force was determined at different steps as the ratio of the systolic pressure (SP, cuff sphygmomanometer)/end-systolic volume index (ESV, biplane Simpson rule/body surface area). Heart rate was determined from ECG., Results: The absolute value of the FFR slope was highest in controls and lowest in DC patients. A flat-downsloping FFR was found in 12/19 patients but not for normals (p<0.01)., Conclusions: Noninvasive pacemaker stress echocardiography (PASE) is a simple and efficient option to assess left ventricular (LV) contractility in patients with permanent pacemaker.

Persistence of left superior vena cava (LSVC) is an uncommon finding during pacemaker implantation, which may be particularly relevant in performing LV transvenous pacing. Rarely, it is further complicated by the presence of atresia of the coronary sinus ostium (CSO). This article reports the authors experience with biventricular pacing (Biv-P) in this unusual clinical setting. From October 1999 to April 2002, 158 patients underwent biventricular pacing. In four of them (mean age 62.2 years), the presence of a persistent LSVC draining into the coronary sinus (CS) was detected at implantation, associated with atresia of the CSO in two patients. A common characteristic was the angiographic finding of a large CS with few tributaries. The LV leads were successfully positioned in the middle cardiac vein in three patients and in a posterolateral vein in one patient. All vessels were large and their cannulation via downstream CS catheterization required the lead to be manipulated through sharp angles. Mean fluoroscopic exposure and procedural times were not significantly different from the overall Biv-P population. In all patients, at a mean follow-up of 11 months, sensing and capture threshold remained stable and a significant decrease in NYHA functional class and increase in LVEF were noted. The direct lead placement in large CS tributaries in the presence of persistent LSVC was feasible and safe. The leads remained stable up to a mean follow-up of nearly 1 year.

Introduction: Inducibility of ventricular arrhythmias at programmed electrical stimulation (PES) ranges between 50% and 80% of patients with Brugada syndrome. However, the variety of PES protocols and the lack of data relative to a control group or to ventricular arrhythmia reproducibility contribute to a still undefined interpretation of PES outcome in Brugada syndrome., Methods and Results: Twenty-one patients with Brugada syndrome (18 men and 3 women; mean age 34 years; 9/21 symptomatic; 8/21 with SCN5A gene mutation) underwent a PES protocol from two right ventricular sites. The endpoint was PES protocol completion or induction of sustained or reproducible (>6 consecutive inductions) nonsustained (>6 beats) fast ventricular arrhythmia. In 17 of 21 patients with Brugada syndrome, PES was repeated 2 months later to test ventricular arrhythmia reproducibility. Twenty-five healthy patients (17 men; mean age 36 years) formed the control group. In patients with Brugada syndrome, ventricular arrhythmia inducibility rate at PES was high (18/21 patients [85%]) and increased with protocol aggressiveness, independent of clinical presentation. In control subjects, no ventricular arrhythmias were induced. Among patients with Brugada syndrome, 14 (82%) of 17 patients remained inducible at a second PES., Conclusion: In our experience, ventricular arrhythmia inducibility in patients with Brugada syndrome, at variance with healthy controls, is high and does not correlate with clinical presentation. PES inducibility is deeply influenced by the protocol used. PES outcome is reproducible at a mid-term follow-up mainly if a categorical endpoint (inducible vs noninducible) is used. The need to assess the predictive value of specific PES protocols in targeted studies is widely emerging and is confirmed by our results.

Background: Mutations in the cardiac ryanodine receptor gene (RyR2) underlie catecholaminergic polymorphic ventricular tachycardia (CPVT), an inherited arrhythmogenic disease occurring in the structurally intact heart. The proportion of patients with CPVT carrying RyR2 mutations is unknown, and the clinical features of RyR2-CPVT as compared with nongenotyped CPVT are undefined., Methods and Results: Patients with documented polymorphic ventricular arrhythmias occurring during physical or emotional stress with a normal heart entered the study. The clinical phenotype of the 30 probands and of 118 family members was evaluated, and mutation screening on the RyR2 gene was performed. Arrhythmias documented in probands were: 14 of 30 bidirectional ventricular tachycardia, 12 of 30 polymorphic ventricular tachycardia, and 4 of 30 catecholaminergic idiopathic ventricular fibrillation; RyR2 mutations were identified in 14 of 30 probands (36% bidirectional ventricular tachycardia, 58% polymorphic ventricular tachycardia, 50% catecholaminergic idiopathic ventricular fibrillation) and in 9 family members (4 silent gene carriers). Genotype-phenotype analysis showed that patients with RyR2 CPVT have events at a younger age than do patients with nongenotyped CPVT and that male sex is a risk factor for syncope in RyR2-CPVT (relative risk=4.2)., Conclusions: CPVT is a clinically and genetically heterogeneous disease manifesting beyond pediatric age with a spectrum of polymorphic arrhythmias. beta-Blockers reduce arrhythmias, but in 30% of patients an implantable defibrillator may be required. Genetic analysis identifies two groups of patients: Patients with nongenotyped CPVT are predominantly women and become symptomatic later in life; patients with RyR2 CPVT become symptomatic earlier, and men are at higher risk of cardiac events. These data provide a rationale for prompt evaluation and treatment of young men with RyR2 mutations.

Optimal management of atrial arrhythmias with dual chamber pacemakers requires proper performance of automatic mode switching (AMS). The aim of this study was to develop a reliable technique to test the AMS function by using an external electronic device capable of mimicking the occurrence of supraventricular arrhythmias (Supraventricular Arrhythmia Simulator [SAS]). The SAS delivers low voltage pulse trains (200 mV, 20 ms) through two skin electrodes. Each pulse train lasts 15 seconds and starts synchronously with a pacing pulse of the implanted pacemaker to avoid interference from the operator. The pulse train rate is set at 350, 250, and 160 beats/min to simulate AF, atrial flutter, and atrial tachycardia (AT), respectively. Thirty-five patients implanted with Vitatron pacemakers, whose AMS system has been previously validated, were enrolled. Atrial and ventricular sensing were programmed in unipolar mode at 0.5 mV and in bipolar mode at > 2 mV, respectively. All pulses from the SAS were detected by the atrial channel at an amplitude ranging from 1 to 3 mV. The test proved to be safe and reliable at rest and during exercise. AMS occurred immediately at onset or at offset of atrial arrhythmias, and no adverse interference on pacemaker function was seen from the SAS. In conclusion, the described technique and the SAS are safe and reliable for patient and pacemaker function and can be proposed as a useful method to verify proper performance of AMS function irrespective of the type of implanted devices.