Maggi L.,Fondazione IRCCS Instituto Neurologico Carlo Besta |
D'Amico A.,IRCCS Bambino Gesu Childrens Hospital |
Pini A.,IRCCS Institute of Neurological science |
Sivo S.,Catholic University |
And 28 more authors.
Neurology | Year: 2014
Objectives: Our aim was to conduct a comparative study in a large cohort of myopathic patients carrying LMNA gene mutations to evaluate clinical and molecular features associated with different phenotypes. Methods: We performed a retrospective cohort study of 78 myopathic patients with LMNA mutation and 30 familial cases with LMNA mutation without muscle involvement. We analyzed features characterizing the various forms of LMNA-related myopathy through correlation statistics. Results: Of the 78 patients, 37 (47%) had limb-girdle muscular dystrophy 1B (LGMD1B), 18 (23%) congenital muscular dystrophy (MDCL), 17 (22%) autosomal dominant Emery-Dreifuss muscular dystrophy 2 (EDMD2), and 6 (8%) an atypical myopathy. The myopathic phenotypes shared a similar cardiac impairment. Cardioverter defibrillator or pacemaker was implanted in 41 (53%) myopathic patients compared to 7 (23%) familial cases without muscle involvement (p = 0.005). Heart transplantation was performed in 8 (10.3%) myopathic patients and in none of the familial cases. Ten (12.8%) myopathic patients died; there were no deaths among the familial cases (p = 0.032). Missense mutations were found in 14 patients (82%) with EDMD2 and 14 patients (78%) with MDCL compared to 17 patients (45%) with LGMD1B and 4 (67%) atypical patients. Frameshift mutations were detected in 17 (45%) LGMD1B compared to 3 (18%) EDMD2, 1 (6%) MDCL, and 2 (33%) with atypical myopathy (p = 0.021). Furthermore, frameshift mutations were found in 30 of 73 patients (41%) with heart involvement compared to 4 of 35 (11%) without heart involvement (p = 0.004). Conclusions: Our data provided new insights in LMNA-related myopathies, whose natural history appears to be dominated by cardiac involvement and related complications. © 2014 American Academy of Neurology.
PubMed | Arrhythmia Unit and Hospital Universitario Central Of Asturias
Type: Journal Article | Journal: Heart rhythm | Year: 2015
Time-dependent variability of electrocardiogram (ECG) in patients with Brugada syndrome could affect the interpretation of provocative testing.The aim of this study was to characterize ECG changes during and after flecainide infusion.We studied 59 consecutive patients. The ECG was continuously analyzed during the first 30 minutes of provocative testing, and a single ECG was recorded 60 minutes later. We analyzed CYP2D6 and CYP3A5 variants affecting flecainide metabolism and performed blinded measurements at lead II.At baseline, ECG patterns were classified as follows: type II in 31 patients (53%), type III in 15 (25%), and normal ECG in 13 (22%). Because of induction of type I ECG, the percentage of responders progressively increased with longer recording time periods (6.8% in 10 minutes vs 11.9% in 20-30 minutes vs 18.6% in 90 minutes; P < .01). Four patients displayed a late response, which was evidenced 90 minutes after the initiation of provocative testing. QRS width differentially increased between responders and nonresponders (P < .01), with a maximum QRS width of 110 ms during the first 30 minutes being effective for identifying possible late responders (sensitivity 100%; specificity 85.6%; positive predictive value 88%; negative predictive value 100%). The incidence of CYP2D6 variants was lower in late responders than in early or delayed responders (0% vs 75% vs 100%; P = .04), while a homogeneous distribution of CYP3A5*3/*3 was observed in our population.Response to flecainide exhibits time-dependent variability of ECG patterns and intervals. Longer periods of ECG recording increase the recognition probability of type I ECG.
Stockburger M.,Charite University Hospital |
Defaye P.,University Hospital |
Boveda S.,Arrhythmia Unit |
Stancak B.,Hospital of Eastern Slovakia |
And 6 more authors.
Europace | Year: 2016
Aims This ANSWER (EvaluAtioN of the SafeR mode in patients With a dual chambER pacemaker indication) sub-study assesses safety and effectiveness of SafeR™ and the impact of ventricular pacing (VP) prevention on anticipated device longevity and replacement rate. Methods and results Patients implanted for atrioventricular block (AVB, n = 310) or sinus node dysfunction (SND, n = 336) were randomly assigned to SafeR (n = 314) or DDD (n = 318) and followed for 36 months. Safety, median VP, estimated device longevity (mean difference, 95% confidence interval [CI]), and anticipated replacement rates were analysed by pacing mode and implant indication. No difference in mortality, syncope, or mode intolerance was observed between randomization groups regardless of the indication. Ventricular pacing on SafeR vs. DDD was 11.5 vs. 93.6% in the overall population (P < 0.001), 89.2 vs. 83.8% in permanent AVB (P = 0.944), 53.5 vs. 98.2% in intermittent AVB (P < 0.001), and 2.2 vs. 84.7% in SND (P < 0.001). Anticipated median device longevity increased on SafeR by 14 [Q1 10; Q3 17] months [10; 17] (P < 0.001) in the overall population, 9 months [-5; 22] (P = 0.193) in permanent AVB, 14 months [8; 19] (P < 0.001) in intermittent AVB, and 14 months [9; 19] (P < 0.001) in SND. In intermittent AVB and SND, prolonged estimated battery longevity translated into the prevention of one anticipated replacement in at least 23% of patients. Conclusion SafeR was effective in reducing VP in intermittent AVB and in SND. No effect was observed in permanent AVB. No safety issue was observed. Ventricular pacing reduction by SafeR translated into relevant estimated prolongation of device longevity and anticipated reduction of required replacements. © The Author 2015.
PubMed | Hospital of Eastern Slovakia, A Pare Private Hospital, Charite University Hospital, Arrhythmia Unit and 5 more.
Type: Journal Article | Journal: Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology | Year: 2016
This ANSWER (EvaluAtioN of the SafeR mode in patients With a dual chambER pacemaker indication) sub-study assesses safety and effectiveness of SafeR and the impact of ventricular pacing (VP) prevention on anticipated device longevity and replacement rate.Patients implanted for atrioventricular block (AVB, n = 310) or sinus node dysfunction (SND, n = 336) were randomly assigned to SafeR (n = 314) or DDD (n = 318) and followed for 36 months. Safety, median VP, estimated device longevity (mean difference, 95% confidence interval [CI]), and anticipated replacement rates were analysed by pacing mode and implant indication. No difference in mortality, syncope, or mode intolerance was observed between randomization groups regardless of the indication. Ventricular pacing on SafeR vs. DDD was 11.5 vs. 93.6% in the overall population (P < 0.001), 89.2 vs. 83.8% in permanent AVB (P = 0.944), 53.5 vs. 98.2% in intermittent AVB (P < 0.001), and 2.2 vs. 84.7% in SND (P < 0.001). Anticipated median device longevity increased on SafeR by 14 [Q1 10; Q3 17] months [10; 17] (P < 0.001) in the overall population, 9 months [-5; 22] (P = 0.193) in permanent AVB, 14 months [8; 19] (P < 0.001) in intermittent AVB, and 14 months [9; 19] (P < 0.001) in SND. In intermittent AVB and SND, prolonged estimated battery longevity translated into the prevention of one anticipated replacement in at least 23% of patients.SafeR was effective in reducing VP in intermittent AVB and in SND. No effect was observed in permanent AVB. No safety issue was observed. Ventricular pacing reduction by SafeR translated into relevant estimated prolongation of device longevity and anticipated reduction of required replacements.
Di Marco A.,Arrhythmia Unit |
Di Marco A.,Bellvitge Universisty Hospital |
Paglino G.,Arrhythmia Unit |
Oloriz T.,Arrhythmia Unit |
And 12 more authors.
Journal of Cardiovascular Electrophysiology | Year: 2015
Introduction In patients with a prior myocardial infarction (MI), angiographic predictors of ventricular tachycardia (VT) recurrence after ablation are lacking. Recently, a proarrhythmic effect of a chronic total occlusion (CTO) in a coronary artery has been suggested. Methods and Results A total of 191 patients with prior MI were referred to our Hospital between 2010 and June 2013 for a first ablation of VT. Of these, 84 patients (44%) with stable coronary artery disease that underwent a coronary angiography during the index hospitalization were included in this study. A CTO in an infarct-related artery (IRA-CTO) was present in 47 patients (56%). Patients with and without IRA-CTO did not differ in terms of comorbidities, severity of heart failure, presentation of VT or acute outcome of ablation, that was completely successful in 93% of cases. At electroanatomic mapping, IRA-CTO was associated with greater scar and especially with greater area of border zone (34 cm2 vs. 19 cm2, P = 0.001). Median follow-up was 19 months (IQR 18). At follow-up, patients with IRA-CTO had a significantly higher rate of VT recurrence (47% vs. 16%, P = 0.003). At multivariate analysis, IRA-CTO resulted to be an independent predictor of VT recurrence after ablation (HR 4.05, P = 0.004). Conclusions IRA-CTO is an independent predictor of VT recurrence after ablation and identifies a subgroup of patients with high recurrence rate despite a successful procedure. IRA-CTO is associated with greater scars and border zone area; however, this association does not completely justify its proarrhythmic effect. © 2015 Wiley Periodicals, Inc.
Kumar S.,Arrhythmia Unit |
Barbhaiya C.R.,Arrhythmia Unit |
Sobieszczyk P.,Interventional Cardiology and Vascular Medicine |
Eisenhauer A.C.,Interventional Cardiology and Vascular Medicine |
And 11 more authors.
Circulation: Arrhythmia and Electrophysiology | Year: 2015
Background - Ventricular tachycardia (VT) refractory to antiarrhythmic drugs and standard percutaneous catheter ablation techniques portends a poor prognosis. We characterized the reasons for ablation failure and describe alternative interventional procedures in this high-risk group. Methods and Results - Sixty-seven patients with VT refractory to 4±2 antiarrhythmic drugs and 2±1 previous endocardial/epicardial catheter ablation attempts underwent transcoronary ethanol ablation, surgical epicardial window (Epi-window), or surgical cryoablation (OR-Cryo; age, 62±11 years; VT storm in 52%). Failure of endo/epicardial ablation attempts was because of VT of intramural origin (35 patients), nonendocardial origin with prohibitive epicardial access because of pericardial adhesions (16), and anatomic barriers to ablation (8). In 8 patients, VT was of nonendocardial origin with a coexisting condition also requiring cardiac surgery. Transcoronary ethanol ablation alone was attempted in 37 patients, OR-Cryo alone in 21 patients, and a combination of transcoronary ethanol ablation and OR-Cryo (5 patients), or transcoronary ethanol ablation and Epi-window (4 patients), in the remainder. Overall, alternative interventional procedures abolished ≥1 inducible VT and terminated storm in 69% and 74% of patients, respectively, although 25% of patients had at least 1 complication. By 6 months post procedures, there was a significant reduction in defibrillator shocks (from a median of 8 per month to 1; P<0.001) and antiarrhythmic drug requirement although 55% of patients had at least 1 VT recurrence, and mortality was 17%. Conclusions - A collaborative strategy of alternative interventional procedures offers the possibility of achieving arrhythmia control in high-risk patients with VT that is otherwise uncontrollable with antiarrhythmic drugs and standard percutaneous catheter ablation techniques. © 2015 American Heart Association, Inc.
Pedrote A.,Arrhythmia Unit |
Arana-Rueda E.,Arrhythmia Unit |
Garcia-Riesco L.,Arrhythmia Unit |
Sanchez-Brotons J.,Arrhythmia Unit |
And 4 more authors.
Journal of Cardiovascular Electrophysiology | Year: 2013
Preablation Atrial Fibrillation Burden: Aims: Data on the success rate of ablation in atrial fibrillation (AF) are controversial. Our hypothesis is that the efficacy must be evaluated considering the AF burden (AFB) before the procedure. Moreover, the clinical significance of early recurrence (ERAT) of AF or atrial tachyarrhythmias (AT) is debatable. The aim is to describe the outcome of pulmonary vein isolation in paroxysmal AF through a subcutaneous cardiac monitor (ICM) implanted before the procedure. Methods: Using CARTO 3, circumferential lesions around the pulmonary veins were placed. The study endpoint was the first documented recurrence of AF/AT by ICM after the blanking period (3 months). AFB (percentage of time in AF/AT) was collected every month before and after the procedure during the 12-month follow-up. Results: The ICM was implanted 94 ± 23 days before the procedure in 35 patients with paroxysmal AF (54 ± 11 years, refractory to 1.8 ± 0.6 antiarrhythmic drugs). Cumulative AFB before the procedure and after the blanking period was 2.5% (1-5%) versus 0% (0-0.25%), P < 0.001. Twenty patients (57.1%) were free of documented AF/AT recurrence, 5 patients (14.2%) reduced the AFB 90%, 6 patients (17.1%) continued the same, and 4 patients (11.4%) increased the AFB 90% for AT. The success rate with second procedure was 71.4%. All 13 patients with ERAT had recurrence after the blanking period. Conclusions: The outcome of pulmonary vein isolation in patients with paroxysmal AF is well documented by an ICM. The success rate is dependent of the previous AFB that can be randomly variable and lower than expected. ERATs predict late recurrence. © 2013 Wiley Periodicals, Inc.
Silberbauer J.,Arrhythmia Unit |
Oloriz T.,Arrhythmia Unit |
MacCabelli G.,Arrhythmia Unit |
Tsiachris D.,Arrhythmia Unit |
And 15 more authors.
Circulation: Arrhythmia and Electrophysiology | Year: 2014
Background-Successful late potential (LP) abolition and postprocedural ventricular tachycardia (VT) noninducibility constitute signifcant end points after catheter ablation for VT. We investigated the prognostic impact of a combined procedural end point of VT noninducibility and LP abolition in a large series of post-myocardial infarction patients with VT. Methods and Results-A total of 160 (154 men, 94% with implantable cardioverter defbrillators) consecutive post- myocardial infarction patients undergoing frst-time ablation procedures from 2010 to 2012 were included. Of the 159 patients surviving the procedure, 137 (86%) were either inducible or in VT at baseline and 103 (65%) had baseline LP presence, of which 79 (77%) underwent successful LP abolition. The combined end point was assessable in 155 (97%) patients. There were 50 (32%) patients with VT recurrences and 17 (11%) cardiac deaths during follow-up. Patients who fulflled the combined end point of VT noninducibility and LP abolition compared with inducible patients exhibited a signifcantly lower incidence of VT recurrence (16.4% versus 47.4%; log-rank P<0.001) and cardiac death (4.1% versus 42.1%; log-rank P<0.001). Among noninducible patients, those with additional LP abolition also had a lower incidence of VT recurrence (16.4% versus 46.0%; log-rank P<0.001). After multivariate analysis, the combined end point of VT noninducibility and LP abolition (hazard ratio, 0.205, P<0.001) was independently associated with VT recurrence and cardiac death (hazard ratio, 0.106; P=0.001). Conclusions-Achieving a combined catheter ablation procedural end point of VT noninducibility and LP abolition reduces VT recurrence rates to low levels (16%). The overall strategy was associated with a signifcant impact on cardiac survival. © 2014 American Heart Association, Inc.