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Introduction: Limited data exist on pulsed-field ablation (PFA) in patients with persistent atrial fibrillation (PeAF) undergoing left atrial posterior wall isolation (LAPWI)., Methods: The Advanced TecHnologies For SuccEssful AblatioN of AF in Clinical Practice (ATHENA) prospective registry included consecutive patients referred for PeAF catheter ablation at 9 Italian centers, treated with the FARAPULSE TM -PFA system. The primary efficacy and safety study endpoints were the acute LAPWI rate, freedom from arrhythmic recurrences and the incidence of major periprocedural complications. Patients undergoing pulmonary vein isolation (PVI) alone, PWI + LAPWI and redo procedures were compared., Results: Among 249 patients, 21.7% had long-standing PeAF, 79.5% were male; mean age was 63 ± 9 years. LAPWI was performed in 57.6% of cases, with 15.3% being redo procedures. Median skin-to-skin times (PVI-only 68 [60-90] vs. PVI + LAPWI 70 [59-88] mins) did not differ between groups. 45.8% LAPWI cases were approached with a 3D-mapping system, and 37.3% with intracardiac echocardiography. LAPWI was achieved in all patients by means of PFA alone, in 88.8% cases at first pass. LAPWI was validated either by an Ultrahigh-density mapping system or by recording electrical activity + pacing maneuvers. No major complications occurred, while 2.4% minor complications were detected. During a median follow-up of 273 [191-379] days, 41 patients (16.5%) experienced an arrhythmic recurrence after the 90-day blanking period, with a mean time to recurrence of 223 ± 100 days and no differences among ablation strategies., Conclusion: LAPWI with PFA demonstrates feasibility, rapidity, and safety in real-world practice, offering a viable alternative for PeAF patients. LAPWI is achievable even with a fluoroscopy-only method and does not significantly extend overall procedural times., (© 2024 The Authors. Journal of Cardiovascular Electrophysiology published by Wiley Periodicals LLC.)

Introduction: Brugada syndrome (BrS) has a dynamic ECG pattern that might be revealed by certain conditions such as fever. We evaluated the incidence and management of ventricular arrhythmias (VAs) related to COVID-19 infection and vaccination among BrS patients carriers of an implantable loop recorder (ILR) or implantable cardioverter-defibrillator (ICD) and followed by remote monitoring., Methods: This was a multicenter retrospective study. Patients were carriers of devices with remote monitoring follow-up. We recorded VAs 6 months before COVID-19 infection or vaccination, during infection, at each vaccination, and up to 6-month post-COVID-19 or 1 month after the last vaccination. In ICD carriers, we documented any device intervention., Results: We included 326 patients, 202 with an ICD and 124 with an ILR. One hundred and nine patients (33.4%) had COVID-19, 55% of whom developed fever. Hospitalization rate due to COVID-19 infection was 2.76%. After infection, we recorded only two ventricular tachycardias (VTs). After the first, second, and third vaccines, the incidence of non-sustained ventricular tachycardia (NSVT) was 1.5%, 2%, and 1%, respectively. The incidence of VT was 1% after the second dose. Six-month post-COVID-19 healing or 1 month after the last vaccine, we documented NSVT in 3.4%, VT in 0.5%, and ventricular fibrillation in 0.5% of patients. Overall, one patient received anti-tachycardia pacing and one a shock. ILR carriers had no VAs. No differences were found in VT before and after infection and before and after each vaccination., Conclusions: From this large multicenter study conducted in BrS patients, followed by remote monitoring, the overall incidence of sustained VAs after COVID-19 infection and vaccination is relatively low., (© 2023 Wiley Periodicals LLC.)

Introduction: The assessment of the ventricular myocardial substrate critically depends on the size of mapping electrodes, their orientation with respect to wavefront propagation, and interelectrode distance. We conducted a dual-center study to evaluate the impact of microelectrode mapping in patients undergoing catheter ablation (CA) of ventricular tachycardia (VT)., Methods: We included 21 consecutive patients (median age, 68 [12], 95% male) with structural heart disease undergoing CA for electrical storm (n = 14) or recurrent VT (n = 7) using the QDOT Micro catheter and a multipolar catheter (PentaRay, n = 9). The associations of peak-to-peak maximum standard bipolar (BV c ) and minibipolar (PentaRay, BV p ) with microbipolar (BV μMax ) voltages were respectively tested in sinus rhythm with mixed effect models. Furthermore, we compared the features of standard bipolar (BE) and microbipolar (μBE) electrograms in sinus rhythm at sites of termination with radiofrequency energy., Results: BV μMax was moderately associated with both BV c (β = .85, p < .01) and BV p (β = .56, p < .01). BV μMax was 0.98 (95% CI: 0.93-1.04, p < .01) mV larger than corresponding BV c , and 0.27 (95% CI: 0.16-0.37, p < .01) mV larger than matching BVp in sinus rhythm, with higher percentage differences in low voltage regions, leading to smaller endocardial dense scar (2.3 [2.7] vs. 12.1 [17] cm 2 , p < .01) and border zone (3.2 [7.4] vs. 4.8 [20.1] cm 2 , p = .03) regions in microbipolar maps compared to standard bipolar maps. Late potentials areas were nonsignificantly greater in microelectrode maps, compared to standard electrode maps. At sites of VT termination (n = 14), μBE were of higher amplitude (0.9 [0.8] vs. 0.4 [0.2] mV, p < .01), longer duration (117 [66] vs. 74 [38] ms, p < .01), and with greater number of peaks (4 [2] vs. 2 [1], p < .01) in sinus rhythm compared to BE., Conclusion: microelectrode mapping is more sensitive than standard bipolar mapping in the identification of viable myocytes in SR, and may facilitate recognition of targets for CA., (© 2023 The Authors. Journal of Cardiovascular Electrophysiology published by Wiley Periodicals LLC.)

Introduction: The prediction of ventricular tachyarrhythmias among patients with implantable cardioverter defibrillators is difficult with available clinical tools. We sought to assess whether in patients with heart failure (HF) and reduced ejection fraction with defibrillators, physiological sensor-based HF status, as summarized by the HeartLogic index, could predict appropriate device therapies., Methods: Five hundred and sixty-eight consecutive HF patients with defibrillators (n = 158, 28%) or cardiac resynchronization therapy-defibrillators (n = 410, 72%) were included in this prospective observational multicenter analysis. The association of both HeartLogic index and its physiological components with defibrillator shocks and overall appropriate therapies was assessed in regression and time-dependent Cox models., Results: Over a follow-up of 25 (15-35) months, 122 (21%) patients received an appropriate device therapy (shock, n = 74, 13%), while the HeartLogic index crossed the threshold value (alert, HeartLogic ≥ 16) 1200 times (0.71 alerts/patient-year) in 370 (65%) subjects. The occurrence of ≥1 HeartLogic alert was significantly associated with both appropriate shocks (Hazard ratios [HR]: 2.44, 95% confidence interval [CI]: 1.49-3.97, p = .003), and any appropriate defibrillator therapies. In multivariable time-dependent Cox models, weekly IN-alert state was the strongest predictor of appropriate defibrillator shocks (HR: 2.94, 95% CI: 1.73-5.01, p < .001) and overall therapies. Compared with stable patients, patients with appropriate shocks had significantly higher values of HeartLogic index, third heart sound amplitude, and resting heart rate 30-60 days before device therapy., Conclusion: The HeartLogic index is an independent dynamic predictor of appropriate defibrillator therapies. The combined index and its individual physiological components change before the arrhythmic event occurs., (© 2023 The Authors. Journal of Cardiovascular Electrophysiology published by Wiley Periodicals LLC.)

Introduction: Lesion Index (LSI) has been developed to predict lesion efficacy during radiofrequency (RF) catheter ablation. However, its value in predicting lesions size has still to be established. The aim of our study was to assess the lesions size reproducibility for prespecified values of LSI reached during RF delivery in an in vivo beating heart., Methods: Ablation lesions were created with different values of LSI in seven domestic pigs by means of a contact force-sensing catheter (TactiCath TM , Abbott). Lesions were identified during RF delivery by means of a three-dimensional mapping system (EnSite TM Precision, Abbott) and measured after heart explantation. Histology was carried out after gross examination on the first three lesions to confirm the accuracy of the macroscopic evaluation., Results: A total of 64 myocardial lesions were created. Thirty-nine lesions were excluded from the analysis for the following reasons: histological confirmation of macroscopic lesion measurement (n = 3), transmurality (n = 24), unfavorable anatomic position (n = 10), not macroscopically identifiable (n = 2). In a final set of 25 nontransmural lesions, injury width and depth were, respectively, 4.6 ± 0.6 and 2.6 ± 0.8 mm for LSI = 4, 7.3 ± 0.8 and 4.7 ± 0.6 mm for LSI = 5, and 8.6 ± 1.2 and 7.2 ± 1.1 mm for LSI = 6. A strong linear correlation was observed between LSI and lesion width (r = .87, p < .00001) and depth (r = .89, p < .00001). Multiple linear regression analysis identified LSI as the only ablation parameter that significantly predicted lesion width (p < .001) and depth (p < .001)., Conclusion: In our in vivo study, LSI proved highly predictive of lesion size and depth., (© 2022 Wiley Periodicals LLC.)

Complete atrioventricular block (CAVB) is a total dissociation between the atrial and ventricular activity, in the absence of atrioventricular conduction. Several diseases may result in CAVB in the pediatric and young-adult population. Permanent right ventricular (RV) pacing is required in permanent CAVB, when the cause is neither transient nor reversible. Continuous RV apical pacing has been associated with unfavorable outcomes in several studies due to the associated ventricular dyssynchrony. This study aims to summarize the current literature regarding CAVB in the pediatric and young adult population and to explore future treatment perspectives., (© 2021 Wiley Periodicals LLC.)

Introduction: The PRAETORIAN score (PS) was developed to assess the implant position and predict defibrillation success of the subcutaneous implantable cardioverter defibrillators (S-ICD). The main critique moved to the routine use of PS has been its postprocedural timing, that limits its usefulness on procedure guidance. The aim of this proof-of-concept study was to assess the feasibility of an intraprocedural use of PS., Methods: Forty consecutive patients undergoing S-ICD implantation were enrolled. Intraprocedural PS (IP-PS) obtained with fluoroscopy before closure of the pocket and postprocedural PS (PP-PS) obtained with two-views chest X-ray were compared. Intraprocedural data and PS were compared with the historic cohorts of the involved institutions., Results: When assessing IP-PS and PP-PS, a complete overall agreement was observed (100%, 1.00-κ; p < .001). When assessing a per-step agreement, a very high-degree of concordance in evaluating Step 1 of the PS was observed (95%, 0.81-κ; p < .001). A complete agreement in Step 2-3 (100%, 1.00-κ; p < .001) of the PS was reported. In comparison with our historical cohort, procedural time in the IP-PS cohort did not increase (45 [41-52] vs. 45 [39-49] min; p = .351) while the expected increase in fluoroscopy time resulted scarce (15 [10-15] s)., Conclusion: An IP-PS can be reliably obtained using fluoroscopy guidance during S-ICD implantation, without a significant increase in procedural duration and may serve as guidance for implanting physicians, to avoid postprocedural S-ICD repositioning, leading to patient discomfort and significantly enhancing infective risks. IP-PS showed a very high agreement with the PP-PS obtained from two-views chest X-ray., (© 2021 Wiley Periodicals LLC.)

Background: Outcomes of catheter ablation (CA) among patients with nonparoxysmal atrial fibrillation (AF) are largely disappointing., Objective: We sought to evaluate the feasibility, effectiveness, and safety of a single-stage stepwise endo-/epicardial approach in patients with persistent/longstanding-persistent AF., Methods: We enrolled 25 consecutive patients with symptomatic persistent (n = 4) or longstanding-persistent (n = 21) AF and at least one prior endocardial procedure, who underwent CA using an endo-/epicardial approach. Our anatomical stepwise protocol included multiple endocardial as well as epicardial (Bachmann's bundle [BB] and ligament of Marshall ablations) components, and entailed ablation of atrial tachycardias emerging during the procedure. The primary outcome was freedom from any AF/atrial tachycardia episode after a 3-month blanking period. The secondary outcome was patients' symptom status during follow-up., Results: The stepwise endo-/epicardial approach allowed sinus rhythm restoration in 72% of patients, either directly (n = 6, 24%) or after AF organization into atrial tachycardia (n = 12, 48%). BB's ablation was commonly implicated in arrhythmia termination. After a median follow-up of 266 days (interquartile range, 96 days), survival free from AF/atrial tachycardia was 88%. Antiarrhythmic drugs could be discontinued in 22 patients (88%). As compared to baseline, more patients were asymptomatic at 9-month follow-up (0% vs. 56%, p = .02). Five patients (20%) developed mild medical complications, whereas one subject (4%) had severe kidney injury requiring dialysis., Conclusion: A single-stage endo-/epicardial CA resulted in favorable rhythm and symptom outcomes in a cohort of patients with symptomatic persistent/longstanding-persistent AF and one or more prior endocardial procedures. Epicardial ablation of BB was commonly implicated in procedural success., (© 2021 The Authors. Journal of Cardiovascular Electrophysiology published by Wiley Periodicals LLC.)

Background: This study examined incidence of AF following cavotricuspid isthmus (CTI) ablation alone or CTI plus prophylactic pulmonary vein isolation (PVI) in patients presenting with isolated atrial flutter (AFL) with no history of AF., Methods and Results: We enrolled 216 patients with isolated typical atrial flutter and randomized them to CTI alone (group 1, n = 108, 61.2 ± 9.7 year, 75% male) or CTI+PVI ablation (group 2, n = 108, 62.4 ± 9.3 year, 73% male). Insertible loop recorder (ILR) was implanted in 21 and 19 patients from groups 1 and 2, respectively. Remaining patients were monitored with event recorders, ECG, 7-day Holter. Follow-up period was for 18 ± 6 months. Compared to group 1, group 2 had significantly longer procedural duration (75.9 ± 33 min vs. 161 ± 48 min [P < 0.001]) and fluoroscopy time (15.9 ± 12.3 min vs. 56.4+21 min [P < 0.001]). At the end of follow-up, 65 (60.2%) in group 1 and 77 (71.3%) in group 2 were arrhythmia free off-AAD (log-rank P = 0.044). A subgroup analysis was performed with 55 year age cut-off. In the <55 age group the CTI only population had similar success as in CTI+PVI, (21 of 24 [83.3%] vs. 19 of 22 [86.4%], respectively, log-rank P = 0.74). In the ≥55 group, having CTI+PVI showed significantly higher success compared to CTI only; 45 of 84 (53.6%) were AF/AT free in CTI only group versus 58 of 86 (67.4%) with CTI+PVI (log-rank P = 0.029)., Conclusion: Prophylactic PVI reduced new-onset AF in patients with lone atrial flutter., (© 2015 Wiley Periodicals, Inc.)

Introduction: It is unclear whether extended pulmonary vein antrum isolation (PVAI) plus nonpulmonary vein (non-PV) trigger elimination prevents more arrhythmia recurrence than PVAI alone in patients with mechanical mitral valve (MMV) undergoing AF ablation. This study compared the efficacy and long-term outcome of 2 ablation strategies--PVAI alone versus extended PVAI plus non-PV trigger elimination--for the treatment of AF in patients with MMV., Methods and Results: One hundred and nine consecutive AF patients with MMV were divided into 2 groups: standard PVAI was performed in group 1 (N = 45); in group 2 (N = 64) PVAI was extended to the LA posterior wall, LA septum, and CS; and all non-PV triggers were eliminated. Patients were followed up for 3 years. At the 12th month, 7 (15.6%) patients in group 1, and 39 (60.9%) patients in group 2 were arrhythmia free (log-rank P < 0.001). Four patients (8.9%; 3 cases of AT and 1 case of AF) from group 1, and 12 patients (18.8%; 9 cases of AT, and 3 cases of AFL) from group 2 experienced very late recurrence. At 36 ± 7 months follow-up, the cumulative recurrence after a single procedure was 42/45 (93.3%) in group 1, and 37/64 (57.8%) in group 2 (log-rank P < 0.001)., Conclusion: Compared with the standard PVAI alone, a strategy including extended PVAI and non-PV trigger elimination is associated with a higher 12-month and long-term arrhythmia-free survival in patients with MMV undergoing AF ablation. Very late recurrence may occur years after the initial procedure with focal AT as the most common type of recurrent arrhythmia., (© 2014 Wiley Periodicals, Inc.)

Introduction: As the population ages, the number of elderly patients with implantable cardiac devices referred for transvenous lead extraction will dramatically increase in Western countries. The safety and effectiveness of lead extraction in elderly patients has not been well evaluated. We report the safety and effectiveness of transvenous lead extraction in octogenarians., Methods and Results: From January 2005 to January 2011, we reviewed data from consecutive patients ≥ 80 years referred to our institutions for transvenous lead extraction because of cardiac device infection or lead malfunction. Clinical characteristics, procedural features, and periprocedural major and minor complications were compared between octogenarians and younger patients. Out of 849 patients undergoing lead extraction in the participating institutions during the study period, 150 (18%) patients were octogenarians (mean age 84 years; range 80-96; 64% males). A significantly higher percentage of octogenarians presented with chronic renal failure (55% vs 26%; P < 0.001), history of malignancy (22% vs 6%; P < 0.001), and chronic obstructive pulmonary disease (46% vs 19%; P < 0.001). Complete lead extraction rates were similar in the 2 age groups (97% in octogenarians vs 96% in patients <80 years; P = 0.39). Periprocedural death occurred in 2 (1.3%) patients ≥80 years and in 5 (0.72%) patients <80 years (P = 0.45 for comparison). No differences in terms of other periprocedural major and minor complications were found between the 2 age groups., Conclusion: Despite presenting with a significantly higher rate of comorbidities, transvenous lead extraction can be performed safely and successfully in octogenarians., (© 2012 Wiley Periodicals, Inc.)

Introduction: Patients with previous ablation for atrial fibrillation (AF) may experience recurrence of perimitral flutter (PMFL). These arrhythmias are usually triggered from sources that may also induce AF. This study aims at determining whether ablation of triggers or completing mitral valve isthmus (MVI) block prevents more arrhythmia recurrences., Methods and Results: Sixty-five patients with recurrent PMFL after initial ablation of long standing persistent AF were included in this study. Thirty-two patients were randomized to MVI ablation only (Group 1) and 33 were randomized to cardioversion and repeat pulmonary vein (PV) isolation plus ablation of non-PV triggers (Group 2). MVI bidirectional block was achieved in all but 1 patient from Group 1. In Group 2, reconnection of 17 PVs was detected in 14 patients (42%). With isoproterenol challenge, 44 non-PV trigger sites were identified in 28 patients (85%, 1.57 sites per patient). At 18-month follow-up, 27 patients (84%) from Group 1 had recurrent atrial tachyarrhythmias, of whom 15 remained on antiarrhythmic drug (AAD); however, 28 patients from Group 2 (85%, P < 0.0001 vs Group 1) were free from arrhythmia off AAD. The ablation strategy used in Group 2 was associated with a lower risk of recurrence (hazard ratio = 0.10, 95% CI 0.04-0.28, P < 0.001) and an improved arrhythmia-free survival (log rank P < 0.0001)., Conclusion: In patients presenting with PMFL after ablation for longstanding persistent AF, MVI block had limited impact on arrhythmia recurrence. On the other hand, elimination of all PV and non-PV triggers achieved higher freedom from atrial arrhythmias at follow-up., (© 2011 Wiley Periodicals, Inc.)

Introduction: Assessment of late gadolinium enhancement (LGE) at cardiac magnetic resonance is often used to detect scar in patients with arrhythmias of right ventricular (RV) origin. Recently, electroanatomic mapping (EAM) has been shown to reliably detect scars corresponding to different cardiomyopathic substrates. We compared LGE with EAM for the detection of scar in patients with arrhythmias of RV origin., Methods and Results: Thirty-one patients with RV arrhythmias and biopsy-proven structural heart disease (18 ARVC and 13 myocarditis), and 5 with idiopathic RV outflow tract arrhythmias underwent LGE analysis and EAM with scar validation through EAM-guided endomyocardial biopsy. EAM scars were present in 23 (64%) patients (all with structural heart disease), whereas LGE was present only in 12 (33%). In 2 cases, EAM provided a false-positive diagnosis of a small scar in the basal perivalvular area. LGE correctly diagnosed EAM scar in 48% of patients, resulting in high positive (92%) but low negative (50%) predictive values. The distribution of LGE was significantly associated with the distribution of EAM scars (P < 0.001 in the free wall, P = 0.003 in the outflow tract, and P = 0.023 in the posterior/inferior wall). Presence of LGE reflected a higher extension of EAM scars (34.4 ± 16.5% vs 7.9 ± 10.1% of the RV area, P < 0.001). At receiver operating characteristic (ROC) analysis, an extension of scar ≥20% of the RV area was the best cut-off value to detect LGE (sensitivity 83%, specificity 92%). Of note, LGE missed 10 of 11 (91%) patients with EAM scars <20% of RV area., Conclusions: LGE is significantly less sensitive than EAM in identifying RV cardiomyopathic substrates. Absence of LGE does not rule out the presence of small scars, and EAM with biopsy should be considered to increase the diagnostic yield., (© 2011 Wiley Periodicals, Inc.)

Introduction: Atrial fibrillation (AF) and diabetes mellitus type 2 (DM2) often coexist; however, a small number of patients with DM2 undergoing catheter ablation (CA) of AF have been included in previous studies. The aim of this study was to evaluate safety and efficacy of ablation therapy in DM2 patients with drug refractory AF., Methods and Results: From January 2005 to September 2006, 70 patients with a diagnosis of DM2 and paroxysmal (n = 29) or persistent (n = 41) AF were randomized to receive either pulmonary vein isolation or a new antiarrhythmic drug treatment (ADT) with a 1-year follow-up. The primary endpoint was the time to first AF recurrence. By Kaplan-Meier analysis, at the end of follow-up, 42.9% of patients in the ADT group and 80% of patients who received a single ablation procedure and were without medications were free of AF (P = 0.001). In the ablation group, a significant improvement in quality-of-life (QoL) scores as compared with ADT group was observed. Six patients in the ADT group (17.1%) developed significant adverse drug effects. Hospitalization rate during follow-up was higher in the ADT group (P = 0.01). The only complication attributable to ablation was one significant access-site hematoma., Conclusion: In patients with DM2, CA of AF provides significant clinical benefits over the ADT and appears to be a reasonable approach regarding feasibility, effectiveness, and low procedural risk.

Introduction: Transseptal puncture (TSP) is the conventional approach to assess the left atrial chamber. This technique has been widely used in interventional cardiology and, in the last years, this approach is mostly applied to electrophysiologic procedures. For atrial fibrillation (AF) ablation, two or more transseptal sheaths are often positioned in the left atrium in the majority of centers, therefore requiring two or more transseptal punctures. Theoretically, double puncture could bear additional risks or could increase the risk of persistence of septal defects. We reported the results of a retrospective analysis of a single transseptal puncture as a simplified approach for positioning multiple catheters in the left atrium during AF ablation., Methods: Between November 2003 and November 2005, 1,150 consecutive patients (mean age 54 +/- 8 years, 75% men) were considered for AF ablation and underwent single transseptal puncture for positioning multiple catheters in the left atrium., Results: In only 6 of 1,150 (0.5%) cases a second transseptal puncture was required to position the ablation catheter in the left atrium. Neither acute complications related to transseptal catheterization such as atrial or aortic perforations, pericardial tamponade, thrombotic formation, or air embolism, nor complications due to the attempts of crossing the septum with the ablation catheter were reported. In all cases, each mapping catheter was properly maneuvered at different sites in the left atrium and at the pulmonary veins. In no patient was a residual septal atrial defect after the transseptal maneuvers detected during a mean follow up of 14 +/- 2 months., Conclusions: This retrospective study on a large cohort of patients shows that single transseptal puncture for positioning two or more catheters in the left atrium for AF ablation is a highly successful and safe maneuver with a very low morbidity in the majority of patients. This may avoid potential complications related to a second transseptal puncture.

The case of a 57-year-old woman with a longstanding history of obstructive hypertrophic cardiomyopathy in dilatative phase and incessant monomorphic ventricular tachycardia is reported. Endocardial recordings and electroanatomic mapping suggested local reentry involving right ventricular apical septum, close to a pacing/defibrillation lead tip. Radiofrequency pulses delivered at this site resulted in definite cure of the arrhythmia. It is possible that fibrotic reaction around pacing/defibrillation lead in a diseased myocardium substrate has contributed to the creation of a reentry circuit.

Introduction: Myotonic dystrophy type 1 (MD1) is the most common muscular dystrophy of adult life. Cardiac involvement is characterized by disorders of atrioventricular conduction, ventricular arrhythmias, and sudden death. Heart rate turbulence (HRT) is a noninvasive risk predictor in patients affected by ischemic heart disease. The aim of our study is to assess the prognostic value of HRT in MD1 patients., Methods and Results: We performed HRT analysis by 24-hour Holter recording to calculate turbulence onset (TO) and turbulence slope (TS) in 29 MD1 patients (mean age 52 +/- 10 years), and in 30 patients (mean age 52 +/- 13 years) with frequent ventricular arrhythmias and structurally normal heart (VANH). An electrophysiological study (EPS) tested ventricular arrhythmias inducibility in 22 MD1 patients. TO was significantly different between MD1 and VANH patients (-1.66 +/- 2.04 and -2.98 +/- 1.79%, respectively, P 0.01), while no difference was observed in TS between MD1 and VANH patients (11.12 +/- 6.46 and 9.12 +/- 6 msec/beat, respectively). On EPS, sustained ventricular arrhythmias (SVA) were induced in six MD1 patients. TO was significantly different in inducible MD1 patients (0.88 +/- 1.95%), as compared with both noninducible (-2.49 +/- 1.43%, P < 0.001) or no eligible to EPS (-1.93 +/- 1.63%, P < 0.005) MD1 patients and to VANH patients (-2.98 +/- 1.79%, P < 0.001)., Conclusions: An impairment of TO, a measure of HRT, suggesting impaired cardiac parasympathetic activity, may be a useful, noninvasive predictor of arrhythmic risk in MD1 patients.

Introduction: Conduction disturbances and arrhythmias characterize the cardiac feature of myotonic dystrophy type 1 (MD1); a myocardial involvement has been suggested as part of the cardiac disease. The aim of our study was to investigate the underlying myocardial alterations using electroanatomic mapping (CARTO) and their possible correlation with genetic and neurological findings., Methods and Results: Right atrial and ventricular CARTO maps were obtained in 13 MD1 patients. Thirteen age-matched patients with paroxysmal supraventricular tachycardia and normal heart served as controls. Unipolar voltage (UNI-v), bipolar voltage (BI-v) amplitudes, bipolar potential duration (Bi-dur), and atrial propagation time (A-pt) were measured. UNI-v and BI-v in interatrial septum, anterolateral atrial wall, and right ventricle outflow tract were lower in MD1 patients than controls (P < 0.001). Bi-dur and A-pt were longer in MD1 patients than controls (P < 0.001, P = 0.046, respectively). A significant relationship was documented between CTG triplets and the percentage of Bi-v <0.5 mV in the atrial anteroseptal region (r = 0.6, P = 0.02)., Conclusions: Altered electroanatomic patterns are present in the right cardiac chambers in MD1 patients. Widespread myocardial alterations, not necessarily limited to the conduction system, may support the presence of a cardiac myopathy as part of the disease.