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      The effect of atrial preference pacing on atrial fibrillation electrophysiological substrate in Myotonic Dystrophy type 1 population

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          Abstract

          P-wave dispersion is a non invasive indicator of intra-atrial conduction heterogeneity producing substrate for reentry, which is a pathophysiological mechanism of atrial fibrillation. The relationship between P-wave dispersion (PD) and atrial fibrillation (AF) in Myotonic dystrophy type 1 (DM1) patients is still unclear. Atrial Preference Pacing (APP) is an efficient algorithm to prevent paroxysmal AF in patients implanted with dual-chamber pacemaker. Aim of our study was to evaluate the possible correlation between atrial preference pacing algorithm, P-wave dispersion and AF burden in DM1 patients with normal cardiac function underwent permanent dual-chamber pacemaker implantation.

          We enrolled 50 patients with DM1 (age 50.3 ± 7.3; 11 F) underwent dual-chamber pacemaker implantation for various degree of atrioventricula block. The study population was randomized following 1 months stabilization period to APP algorithm features programmed OFF or ON. Patients were assessed every 3 months for the first year, and every 6 months thereafter up to 3 years. At each follow-up visit, we counted: the number of premature atrial beats, the number and the mean duration of AF episodes, AF burden and the percentage of atrial and ventricular pacing.

          APP ON Group showed lower number of AF episodes (117 ± 25 vs. 143 ± 37; p = 0.03) and AF burden (3059 ± 275 vs. 9010 ± 630 min; p < 0.04) than APP OFF Group. Atrial premature beats count (44903 ± 30689 vs. 13720 ± 7717 beats; p = 0.005) and Pwave dispersion values (42,1 ± 11 ms vs. 29,1 ± 4,2 ms, p = 0,003) were decreased in APP ON Group. We found a significant positive correlation between PD and AF burden (R = 0,8, p = 0.007).

          Atrial preference pacing algorithm, decreasing the number of atrial premature beats and the P-wave dispersion, reduces the onset and perpetuator factors of AF episodes and decreases the AF burden in DM1 patients underwent dual chamber pacemaker implantation for various degree of atrioventricular blocks and documented atrial fibrillation.

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          Most cited references52

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          Characteristics and possible mechanism of ventricular arrhythmia dependent on the dispersion of action potential durations.

          The arrhythmogenic role of increased dispersion of repolarization (dispersion) was studied in 23 open-chest dogs using six simultaneously recorded monophasic action potentials (MAPs) from the ventricular surface and programmed ventricular premature stimulation (VPS). Increased dispersion was induced by generalized hypothermia (29 degrees C) and regional warm blood (38-43 degrees C) perfusion through a coronary artery branch. Hypothermia and regional warm blood perfusion increased maximum dispersion from 13 +/- 10 to 111 +/- 16 msec (p less than 0.001), predominantly because of the increased MAP duration difference (10 +/- 15 vs 97 +/- 16 msec, p less than 0.001). The maximal difference between activation times was not significantly changed, but the QRS duration increased from 47 +/- 6 to 52 +/- 7 msec (p less than 0.01). Ventricular arrhythmia did not occur spontaneously but was induced by a single VPS in all 23 dogs during hypothermia and regional warm blood perfusion when dispersion reached a critical magnitude. The critical magnitude of dispersion required to induce ventricular arrhythmia was documented in 16 dogs by stepwise increments or decrements of dispersion. In four dogs, an increase in atrial pacing rate of 24 beats/min prevented induction of ventricular arrhythmia by decreasing dispersion from a critical magnitude of 103 +/- 5 msec to a nonarrhythmogenic value of 86 +/- 9 msec (p less than 0.05). In six dogs, we compared the stimulation site-dependent effects of VPS applied in the region with short and long MAPs. In all dogs, ventricular arrhythmia was inducible only by VPS from the region with a short MAP. Premature impulses from this region propagated more slowly than those from the region with a long MAP. Our results show that the large dispersion of repolarization facilitates the development of a conduction delay necessary to induce sustained arrhythmia by an early premature stimulus applied at the site with a short MAP.
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            Pathology of the cardiac conduction system in myotonic dystrophy: a study of 12 cases.

            In 12 autopsy cases of myotonic dystrophy, the most frequently observed histopathologic lesions of the cardiac conduction system were fibrosis, fatty infiltration and atrophy. Fibrosis involved the sinus node in 6 cases, atrioventricular (AV) node in 7, AV bundle in 8, bundle branches in 10 and ventricular myocardium in 11. Fatty infiltration was observed in the sinus node in two cases, AV node in two, AV bundle in six, bundle branches in one and ventricular myocardium in nine. Atrophy was prominent in the AV bundle in five and bundle branches in eight. Lymphocytes infiltrated the conduction system in three cases and were associated with myotonic dystrophy in two and varicella myocarditis in one. Ventricular myocytes were hypertrophied in seven cases, vacuolated in three and exhibited disarray in two. The distribution and extent of conduction system lesions tended to correspond to antemortem electrocardiographic abnormalities, including prolonged PR interval in six cases, intraventricular conduction delay in six and bundle branch block in four. Cardiac involvement by myotonic dystrophy may have contributed to sudden death in four cases.
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              Dispersion of repolarization and beta-thalassemia major: the prognostic role of QT and JT dispersion for identifying the high-risk patients for sudden death.

              Patients with beta-thalassemia major (β-TM) are at increased risk for sudden cardiac death (SCD). Heterogeneity of ventricular repolarization is considered to provide an electrophysiological substrate for malignant arrhythmias. QT dispersion (QTc-D) and JT dispersion (JTc-D) are electrocardiographic parameters indicative of heterogeneity of ventricular repolarization. The aim of our study was to evaluate the heterogeneity of ventricular repolarization in patients with beta-thalassemia and to test the hypothesis that an abnormal QTc and JTc dispersion may predict SCD in this population.  The study involved 51 patients with β-TM (age 33.9±8.4; 33M) and 51 healthy subjects used as controls, matched for age, gender, and body mass index (BMI). Among the β-TM group, 14 patients with β-TM (age 27±6.64; 11M) died from SCD during follow-up. For each patient, QTD and JTD intervals were calculated. Compared to the healthy control group, β-TM group presented increased values of the QTc-D (65.36±33.95 vs. 37, 62±17.65; P<0.003) and JTc-D (74.64±33.27 vs. 40.32±12.45; P<0.001). In the β-TM sudden death group, QTc-D and JTc-D were significantly greater than in survived β-TM group (92.70±44.24 vs. 56.14±23.80, P=0.0001; 101.54±47.93 vs. 64.47±17.90, P=0.0001). A cutoff value of 70ms for QTc-D had a sensitivity and specificity of 77% in identifying patients at risk for SCD. A cutoff value of 100ms for JTc-D had a sensitivity of 65% and a specificity of 94% in identifying this category of patients. β-TM is associated with significant changes in heterogeneity of ventricular repolarization. QTc and JTc dispersion are useful markers of risk of SCD in patients with β-TM. © 2011 John Wiley & Sons A/S.
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                Author and article information

                Journal
                Acta Myol
                Acta Myol
                Pacini
                Acta Myologica
                Pacini Editore SpA
                1128-2460
                1128-2460
                December 2014
                : 33
                : 3
                : 127-135
                Affiliations
                [1 ] Chair of Cardiology, Second University of Napoli, Monaldi Hospital, Napoli, Italy;
                [2 ] Cardiology Department, "Luigi Sacco" Hospital, Milano, Italy;
                [3 ] Cardiomyology and Medical Genetics, Department of Experimental Medicine, Second University of Napoli, Italy
                Author notes
                Address for correspondence: Gerardo Nigro, Department of Cardio-Thoracic and Respiratory Sciences, Chair of Cardiology, Second University of Naples, Monaldi Hospital, piazzale Ettore Ruggeri, 80100 Naples, Italy. E-mail: gerardo.nigro@ 123456unina2.it
                Article
                Pacini
                4369849
                25873781
                73f728ce-2551-4376-b694-5b43198e879d
                The journal and the individual contributions contained in it are protected by the copyright of Gaetano Conte Academy, Naples, Italy

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial No Derivatives License, which permits for noncommercial use, distribution, and reproduction in any digital medium, provided the original work is properly cited and is not altered in any way. For details, please refer to http://creativecommons.org/licenses/by-nc-nd/3.0/

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                Original Articles

                Plant science & Botany
                atrial fibrillation,myotonic dystrophy,atrial preference pacing
                Plant science & Botany
                atrial fibrillation, myotonic dystrophy, atrial preference pacing

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