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ABSTRACT: Atrial Preference Pacing (APP) is a pacemaker (PM) algorithm that works by increasing the atrial pacing rate to achieve continuous suppression of a spontaneous atrial rhythm and prevent supraventricular tachyarrhythmias. We have previously shown that atrial preference pacing may significantly reduce the number and the duration of AF episodes in myotonic dystrophy type 1 (DM1) patients who are paced for standard indications.However, the role that APP therapies play in the prevention of AF in a long-term period remains still unclear. Aim of the present prospective study was to evaluate whether this beneficial effect is maintained for 24-months follow-up period.To this aim, 50 patients with Myotonic Dystrophy type 1 who underwent dual-chamber PM implantation for first- and second- degree atrioventricular block, were consecutively enrolled and followed for 2 years. One month later the stabilization period, after the implantation, they were randomized to APP algorithm programmed OFF or ON for 6 months each, using a cross-over design, and remained in the same program for the second year. The results showed that while the number of AF episodes during active treatment (APP ON phases) was lower than that registered during no treatment (APP OFF phases), no statistically significant difference was found in AF episodes duration between the two phases. Furthermore, during the APP OFF and APP ON phases, the percentage of atrial pacing was 0 and 99%, respectively, while the percentage of ventricular pacing did not show differences statistically significant (11 vs. 9%, P = 0.2). Atrial premature beats were significantly higher during APP OFF phases than during APP ON phases. Lead parameters remained stable over time and there were no lead-related complications. Based on these 24-months follow-up data, we can conclude that, in DM1 patients who underwent dual-chamber PM implantation, APP is an efficacy algorithm for preventing paroxysmal AF even in long term periods.
Acta myologica: myopathies and cardiomyopathies: official journal of the Mediterranean Society of Myology / edited by the Gaetano Conte Academy for the study of striated muscle diseases 10/2012; 31(2):139-43.
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ABSTRACT: Background: The development of malignant ventricular arrhythmias is a possible feature in Emery-Dreifuss muscular dystrophy (EDMD) patients with normal left ventricular systolic function. This event may be the cause of sudden cardiac death in EDMD patients. QTc dispersion (QTc-D), JTc dispersion (JTc-D) and Tpeak-end dispersion (TDR) could reflect the physiological variability of regional and transmural ventricular repolarisation and could provide a substrate for life-threatening ventricular arrhythmias. Aim: The current study was designed to evaluate the heterogeneity of ventricular repolarisation in EDMD patients. Methods: Echocardiograms and electrocardiograms from 40 EDMD patients (age 20 ± 13) were evaluated and compared to those of 40 healthy age-matched controls. Results: The EDMD group, compared to the healthy control group, presented increased values of QTc-D (82.8 ± 44.1 vs. 53.3 ± 13.9, p = 0.003), JTc-D (73.6 ± 32.3 vs. 60.4 ± 11.1 ms, p = 0.001) and TDR (100.54 ± 19.06 vs. 92.15 ± 15.5 ms, p = 0.004). No correlation between QTc dispersion and ejection fraction (R = 0.2, p = 0.3) was found. Conclusions: EDMD is associated with significantly increased regional and transmural heterogeneity of ventricular repolarisation, in the absence of impaired systolic and diastolic cardiac function.
Kardiologia polska 01/2012; 70(11):1154-9. · 0.51 Impact Factor
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Vincenzo Russo,
Anna Rago, Alberto Palladino,
Andrea Antonio Papa,
Federica Di Meo,
Nadia Della Cioppa,
Paolo Golino,
Maria Giovanna Russo,
Raffaele Calabrò,
Luisa Politano,
Gerardo Nigro
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ABSTRACT: Paroxysmal episodes of atrial fibrillation frequently occur in Emery-Dreifuss muscular dystrophy (EDMD). Although previous studies have documented a variety of electrocardiographic abnormalities in EDMD, little is still known about P-wave dispersion (PD), an independent risk factor for the development of atrial fibrillation. The aim of our study was to evaluate the P-wave duration and PD in patients with EDMD with conserved systolic and diastolic cardiac function.
The study involved 36 patients with EDMD (age, 20 [SD, 12] years; 26 men) and 36 healthy subjects used as controls, matched for age and sex. P-wave dispersion was carefully measured using 12-lead electrocardiogram. Compared with the healthy control group, patients with EDMD presented increased maximum P-wave duration (108.2 [SD, 22.2] vs 97.8 [SD, 11] milliseconds, P = 0.04) and PD (51.4 [SD, 12.8] vs 39.3 [SD, 9.7] milliseconds, P = 0.004) values. No statistically significant differences in left atrium diameter (37.1 [SD, 2.9] vs 34.1 [SD, 4.2] mm, P = 0.3) and maximum left atrium volume (15.2 [SD, 3.8] vs 14.1 [SD, 4.2] mL/m2, P = 0.4) were found between the 2 groups. We divided our study population into 2 subgroups, according to the different genetic diagnosis, patients with laminopathy EDMD (n = 17) or with emerinopathy EDMD (n = 19). No statistically significant differences were found in PD between the 2 subgroups (54.6 [SD, 15.6] vs 50.2 [SD, 11.5] milliseconds, P = 0.4).
Our study showed a significant increase of maximum P-wave duration and PD in patients with EDMD with conserved systolic and diastolic cardiac function.
Journal of Investigative Medicine 10/2011; 59(7):1151-4. · 1.96 Impact Factor
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Vincenzo Russo,
Anna Rago, Alberto Palladino,
Andrea Antonio Papa,
Federica Di Meo,
Nadia Della Cioppa,
Paolo Golino,
Maria Giovanna Russo,
Raffaele Calabrò,
Luisa Politano,
Gerardo Nigro
[show abstract]
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ABSTRACT: Background: Paroxysmal episodes of atrial fibrillation frequently occur in Emery-Dreifuss muscular dystrophy (EDMD). Although previous studies have documented a variety of electrocardiographic abnormalities in EDMD, little is still known about P-wave dispersion (PD), an independent risk factor for the development of atrial fibrillation. The aim of our study was to evaluate the P-wave duration and PD in patients with EDMD with conserved systolic and diastolic cardiac function.
Methods: The study involved 36 patients with EDMD (age, 20 [SD, 12] years; 26 men) and 36 healthy subjects used as controls, matched for age and sex. P-wave dispersion was carefully measured using 12-lead electrocardiogram. Compared with the healthy control group, patients with EDMD presented increased maximum P-wave duration (108.2 [SD, 22.2] vs 97.8 [SD, 11] milliseconds, P = 0.04) and PD (51.4 [SD, 12.8] vs 39.3 [SD, 9.7] milliseconds, P = 0.004) values. No statistically significant differences in left atrium diameter (37.1 [SD, 2.9] vs 34.1 [SD, 4.2] mm, P = 0.3) and maximum left atrium volume (15.2 [SD, 3.8] vs 14.1 [SD, 4.2] mL/m2, P = 0.4) were found between the 2 groups. We divided our study population into 2 subgroups, according to the different genetic diagnosis, patients with laminopathy EDMD (n = 17) or with emerinopathy EDMD (n = 19). No statistically significant differences were found in PD between the 2 subgroups (54.6 [SD, 15.6] vs 50.2 [SD, 11.5] milliseconds, P = 0.4).
Conclusions: Our study showed a significant increase of maximum P-wave duration and PD in patients with EDMD with conserved systolic and diastolic cardiac function.
Journal of Investigative Medicine 09/2011; 59(7):1151-1154. · 1.96 Impact Factor
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ABSTRACT: To investigate why myotonic dystrophy type 1 (DM1) patients have low intraocular pressure (IOP).
Prospective, comparative case series.
One hundred two eyes of 51 patients with DM1 (age range, 21-64 years) and 44 eyes of 22 healthy subjects of similar age (21-64 years).
All participants underwent IOP measurement with Goldmann applanation tonometry and an in vivo examination of the ciliary body with a 35-MHz high-resolution B-scan. The findings were compared between the 2 groups. In both groups, only patients with no history of ocular trauma or surgery were included. The differences were evaluated using the unpaired Student t test.
Intraocular pressure, central corneal thickness (CCT), and echographic evidence of ciliary body detachment.
The mean ± standard deviation (SD) IOP in patients with DM1 was 10.9 ± 3.1 mmHg and that in the control patients was 15.4 ± 2.2 mmHg, a difference that reached significance (P<0.01). The mean ± SD CCT (measured at the pupillary center) was 574.4 ± 37.9 μm in the patients with DM1 and 557.8 ± 39.2 μm in the controls (P = 0.02). Detachment of the ciliary body was identified in all DM1 subjects. Size was variable and the detachment involved 1 or more quadrants. The number of quadrants affected by the detachment was not correlated with the IOP (R(2) = 0.088) or the size of the CTG expansion. No detachments were found in the healthy controls.
Detachment of the ciliary body may explain the low IOP values in patients with DM1. The finding of a ciliary body detachment in an individual who has not had recent eye surgery or trauma raises the possibility of a DM1 diagnosis.
Ophthalmology 02/2011; 118(2):260-4. · 5.45 Impact Factor
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ABSTRACT: We report the case of 14-year-old boy with X-linked Emery-Dreifuss muscular dystrophy who developed sick sinus syndrome and required placement of an implantable intracardiac cardioverter-defibrillator (ICD) to prevent sudden death. He demonstrated no significant risk factors for sudden death such as depressed left ventricular ejection fraction, or spontaneous or inducible ventricular tachycardia. One month after implantation, the patient experienced one appropriate ICD discharge.
Neuromuscular Disorders 02/2010; 20(3):174-7. · 2.80 Impact Factor
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Gerardo Nigro,
Vincenzo Russo,
Luisa Politano,
Nadia Della Cioppa,
Donatella Manfredi,
Raffaele Chianese,
Annabella De Chiara,
Anna Rago,
Giulia Arena, Alberto Palladino,
Raffaele Calabrò
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ABSTRACT: We performed a two-year follow-up comparative study of long-term electrical parameters between the right atrial appendage (RAA) and Bachmann's Bundle (BB) stimulation in myotonic dystrophy type 1 (MD1) patients.
Twenty-five MD1 patients (18 men; age: 54 +/- 13 years) with no difference in the electrical parameters between the RAA site and the BB region at implantation were randomized into two groups: in group I (13 patients; age: 52 +/- 14 years; four women) the atrial lead was placed in the RAA and in group II (12 patients, age: 56 +/- 12 years, three women) the lead was placed in the BB region. Measurements of electrical parameters were recorded at follow-up intervals of 6 weeks and then 12 and 24 months postimplant.
There was no statistically significant different in P-wave amplitude, pacing threshold, and impedance values between the two groups at 6 weeks. At 24 months follow-up, the intrinsic P-wave amplitude was 2.05 +/- 1.45 mV in the RAA group versus 3.28 +/- 1.09 mV in the BB group (P < 0.05); the pacing threshold was 1.85 +/- 1.8 V in the RAA group versus 0.50 +/- 0.39 V in the BB group (P = 0.03); there were no differences in the atrial impedance between the two groups during the follow-up period.
In a direct two-year follow-up comparison between the RAA and BB atrial pacing sites, we showed a statistically significant increased pacing threshold and decreased intrinsic P-wave amplitude during RAA stimulation in MD1 patients.
Pacing and Clinical Electrophysiology 09/2009; 32(9):1191-6. · 1.35 Impact Factor
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P Cudia,
P Bernasconi,
R Chiodelli,
F Mangiola,
F Bellocci,
A Dello Russo,
C Angelini,
V Romeo,
P Melacini,
L Politano, A Palladino,
G Nigro,
G Siciliano,
M Falorni,
M G Bongiorni,
C Falcone,
R Mantegazza,
L Morandi
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ABSTRACT: To examine the association between the presence of arrhythmia in type 1 myotonic dystrophy (DM1) and clinical-genetic variables, evaluating their role as predictors of the risk of arrhythmia.
245 patients with genetically proven DM1 underwent clinical and non-invasive cardiological evaluation. Severity of muscular involvement was assessed according to the 5 point Muscular Disability Rating Score (MDRS). Data were analysed by univariate and multivariate models.
245 patients were examined and cardiac arrhythmias were found in 63 subjects, 40 of whom required a device implant. Statistical analyses revealed that men had more than double the risk of developing arrhythmias compared with women (p = 0.018). Addition of each year of age caused an increased risk of arrhythmia equal to 3% (p = 0.030). Subjects with MDRS 5 had a risk of arrhythmia 12 times higher than patients with MDRS 1-2 (p<0.001). Although all of these variables were significantly associated with cardiac rhythm dysfunction, they had a low sensitivity for the prediction of arrhythmic risk
Male sex, age and muscular disability were strongly associated with the development of arrhythmia in DM1. However, all of these variables were weak predictors of arrhythmic risk. These results suggest that other factors may be involved in the development of cardiac conduction abnormalities in DM1.
Journal of neurology, neurosurgery, and psychiatry 03/2009; 80(7):790-3. · 4.87 Impact Factor
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Antonio Dello Russo,
Fortunato Mangiola,
Paolo Della Bella,
Giovanni Nigro,
Paola Melacini,
Maria Grazia Bongiorni,
Claudio Tondo,
Leonardo Calò,
Loredana Messano,
Manuela Pace, [......],
Tommaso Sanna,
Gabriella Silvestri,
Anna Modoni,
Elisabetta Zachara,
Massimo Moltrasio,
Lucia Morandi,
Gerardo Nigro,
Luisa Politano, Alberto Palladino,
Fulvio Bellocci
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ABSTRACT: Myotonic dystrophy type 1 (DM1) is the most frequent muscular dystrophy in adults. DM1 is a multisystem disorder also affecting the heart with an increased incidence of sudden death, which has been explained with the common impairment of the conduction system often requiring pacemaker implantation; however, the occurrence of sudden death despite pacemaker implantation and the observation of major ventricular arrhythmias generated the hypothesis that ventricular arrhythmias may play a causal role as well. The aim of the study was to assess the 2-year cumulative incidence and the value of noninvasive and invasive findings as predictive factors for sudden death, resuscitated cardiac arrest, ventricular fibrillation, sustained ventricular tachycardia and severe sinus dysfunction or high-degree atrioventricular block.
More than 500 DM1 patients will be evaluated at baseline with a clinical interview, 12-lead ECG, 24-h ECG and echocardiogram. Conventional and nonconventional indications to electrophysiological study, pacemaker, implantable cardioverter defibrillator or loop recorder implantation have been developed. In the case of an indication to electrophysiological study, pacemaker, implantable cardioverter defibrillator or loop recorder implant at baseline or at follow-up, the patient will be referred for the procedure. At the end of 2-year follow-up, all candidate prognostic factors will be tested for their association with the endpoints.
ClinicalTrials.gov ID NCT00127582.
The available evidence supports the hypothesis that both bradyarrhythmias and tachyarrhythmias may cause sudden death in DM1, but the course of cardiac disease in DM1 is still unclear. We expect that this large, prospective, multicenter study will provide evidence to improve diagnostic and therapeutic strategies in DM1.
Journal of Cardiovascular Medicine 02/2009; 10(1):51-8. · 1.51 Impact Factor
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ABSTRACT: To compare intraocular pressure (IOP) between patients with myotonic dystrophy (DM1) and normal subjects, taking into account corneal characteristics. To determine whether lower IOP measurements in patients with DM1 are due to thinner corneas.
Comparative case series.
Fifty-three eyes of patients with DM1 and 53 eyes of normal age- and sex-matched subjects.
Corneal biomechanical properties and corneal compensated intraocular pressure (IOPcc) measured with the Ocular Response Analyzer (Reichert Inc., Depew, NY), central corneal thickness measured with the Oculus Pentacam (Oculus, Wetzlar, Germany), and IOP were evaluated in patients with DM1 and compared with age- and sex-matched healthy subjects.
Goldmann applanation tonometry, central corneal thickness, corneal hysteresis (CH), corneal resistance factor (CRF), and IOPcc.
Compared with the healthy subjects, patients with DM1 showed lower IOP (12.4+/-3.6 mm Hg vs. 14.9+/-3.4 mmHg) (P<0.01) and IOPcc (12.7+/-4.5 vs. 15.9+/-3.5) (P<0.01), and thicker cornea (575.9+/-35.02 mum vs. 556.3+/-33.2 microm) (P<0.01), but no significant changes in CH (P = 0.03) and CRF (P = 0.37).
Lower IOP in patients with DM1 is not related to differences in central corneal thickness or corneal biomechanical properties.
The author(s) have no proprietary or commercial interest in any materials discussed in this article.
Ophthalmology 11/2008; 116(2):231-4. · 5.45 Impact Factor
