Sinus bradycardia, junctional rhythm, and low-rate atrial fibrillation in Short QT syndrome during 20 years of follow-up: three faces of the same genetic problem

2015 ◽  
Vol 26 (3) ◽  
pp. 589-592 ◽  
Author(s):  
Daniela Righi ◽  
Massimo S. Silvetti ◽  
Fabrizio Drago
Keyword(s):  
Atrial Fibrillation ◽  
Sinus Bradycardia ◽  
Sinus Node ◽  
Sinus Node Dysfunction ◽  
Short Qt Syndrome ◽  
Junctional Rhythm ◽  
Genetic Problem ◽  
Qt Syndrome ◽  
Low Rate

AbstractWe describe the case of an asymptomatic girl with sinus bradycardia and short QT interval at birth, junctional bradycardia in infancy requiring single-chamber pacemaker, atrial fibrillation in adolescence, and V141M mutation in the KCNQ1 gene. Atrial fibrillation recurred and became unresponsive to electrical or anti-arrhythmic therapy. During 20 years of follow-up, a progressive evolution from sinus node dysfunction to low-rate atrial fibrillation was observed.

Abstract 15680: Sinus Node Dysfunction in Associated With Higher Symptom Burden and Increased Risk of Progression to Permanent Atrial Fibrillation: Results From ORBIT-AF Registry

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Larry R Jackson ◽  
Sung Hee Kim ◽  
Jonathan P Piccini ◽  
Bernard J Gersh ◽  
Gerald V Naccarelli ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Atrial Fibrillation ◽  
Sinus Bradycardia ◽  
Sinus Node ◽  
Sinus Node Dysfunction ◽  
Left Ventricular Ejection ◽  
Increased Risk ◽  
Risk Of Progression

Background: Patients with sinus node dysfunction (SND) are at increased risk of atrial tachyarrhythmias, including atrial fibrillation (AF). Whether the presence of SND is also associated with worse outcomes among those with AF has not been well described. Methods: The ORBIT-AF registry enrolled patients with AF from a range of clinical practices across the US. SND was defined clinically, based on the presence of sinus bradycardia, severe sinus bradycardia, sinus arrest, sinoatrial exit block, or features of tachycardia-bradycardia syndrome. Descriptive statistics and multivariable logistic regression analysis were used to describe treatment patterns and outcomes for patients with and without SND and AF. Results: Overall, 1,710 (17.7%) patients had SND at enrollment. Patients with SND had lower left-ventricular ejection fractions, higher CHA 2 DS 2 -VASc risk scores, and more prior cerebrovascular events. Patients with SND had more severe symptoms (EHRA class IV: 17.5% vs. 13.9%; p=0.007) and poorer quality of life (median AFEQT 77.5 vs. 81.1; p=0.008) as compared to those without. SND patients were more frequently treated with oral anticoagulants (79.2% vs. 75.9%, p=0.004) and had more often received interventional therapy for AF (16.1% vs. 10.5%, p<0.0001). There were no differences in the current AF management strategy between patients with SND and those without [rate control (69.7% vs. 67.7%), rhythm control (30.0% vs. 32.0%); P=0.11]. After adjustment, significantly more patients with SND had progressed from paroxysmal AF at baseline to persistent or permanent AF at any follow-up or persistent AF at baseline to permanent AF at any follow-up than those without (OR 1.23, 95% CI 1.01-1.49, p=0.035). Conclusion: Sinus node dysfunction is associated worse symptoms, lower quality of life, and higher risk of progression to permanent AF. However, SND is not associated with increased risk of all-cause hospitalization, incident stroke, or all-cause death.

scholarly journals Comparative characteristics of a pacemaker implantation after biatrial or left atrial ablation of atrial fibrillation in combination with coronary artery bypass grafting in patients with ischemic heart disease and long-standing persistent atrial fibrillation

Kardiologiia ◽  
2021 ◽  
Vol 61 (10) ◽  
pp. 46-52
Author(s):  
A. T. Kalybekova ◽  
S. S. Rakhmonov ◽  
V. L. Lukinov ◽  
A. M. Chernyavsky
Keyword(s):  
Atrial Fibrillation ◽  
Surgical Treatment ◽  
Postoperative Period ◽  
Left Atrial ◽  
Sinus Node ◽  
Persistent Atrial Fibrillation ◽  
Sinus Node Dysfunction ◽  
Av Block ◽  
Atrial Ablation

Aim      To compare the incidence of a permanent pacemaker (PP) implantation based on the chosen treatment technology (biatrial ablation, BA, or left atrial ablation (LAA) for long-standing persistent atrial fibrillation (AF) with simultaneous coronary bypass (CB).Material and methods  The study included 116 patients with long-standing persistent AF and indications for CB. Patients were randomized to two equal groups (58 patients in each). Group 1 underwent BA in combination with CB; group 2 patients underwent isolated LAA with simultaneous CB under the conditions of artificial circulation. Incidence of PP implantation was assessed during the early (to 30 days) and late (to 60 months) postoperative periods.Results For the observation period, a total of 9 PPs was implanted in both groups, 6 in the BA group and 3 in the LAA group (odds ratio, OR, 0.5; 95 % confidence interval, CI, 0.1–2.4; р=0.490). During the early postoperative period, 5 patients in the BA group and 2 patients in the LAA group were implanted with PP (OR, 0.4; 95 % CI. 0–2.5; р=0.438). During the late postoperative period, one (2%) patient of the BA group was implanted with a permanent PP at 30 months of follow-up due to the development of sick sinus syndrome (SSS); also, one (2%) patient of the LAA group required PP implantation at 54 months of follow-up due to the development of SSS. The causes for PP implantation in the BA group included the development of complete atrioventricular (AV) block in 9 % of cases (95 % CI, 4–19 %); sinus node dysfunction and junctional rhythm in 2 % of cases (95 % CI, 0–9 %). Compared to this group, the LAA group showed a statistically significant difference in the incidence of AV block (0 cases, р=0.047). The major cause for PP implantation in the LAA group was the development of sinus node dysfunction in 3 (5 %) patients (95 % CI, 2–14 %).Conclusion      The use of BA in surgical treatment of long-standing persistent AF with simultaneous myocardial revascularization is associated with a high risk of AV block, which requires permanent PP implantation in the postoperative period. Total incidence of permanent PP implantation for dysfunction of the cardiac conduction system following the combination surgical treatment of long-standing persistent AF and IHD, either CB and LAA or BA, did not differ between the treatment groups both in early and late postoperative periods. 

Clinical outcomes with ventricular pacing or dual-chamber pacing for sinus-node dysfunction: a nationwide matched control study

EP Europace ◽  
2021 ◽  
Vol 23 (Supplement_3) ◽  
Author(s):  
L Fauchier ◽  
A Bodin ◽  
A Bisson ◽  
J Herbert ◽  
V Ah-Fat ◽  
...  
Keyword(s):  
Heart Failure ◽  
Atrial Fibrillation ◽  
Clinical Outcomes ◽  
Sinus Node ◽  
Cardiovascular Death ◽  
Sinus Node Dysfunction ◽  
Dual Chamber ◽  
Onset Atrial Fibrillation ◽  
New Onset

Abstract Funding Acknowledgements Type of funding sources: None. Introduction.Evidence from randomized trials suggests that, in patients with sinus-node dysfunction (SND), physiologic pacing (dual-chamber, DDD) may be superior to single-chamber (ventricular, VVI) pacing because it is associated with lower risks of atrial fibrillation and stroke, better exercise capacity and lower risk of pacemaker syndrome. However, benefits on mortality and risk of heart failure have not been demonstrated and these issues have not been fully evaluated in large "real life" analyses. Objective.The aim of our study was to assess and compare clinical outcomes within the first 30 days and during a longer-term follow-up with the two types of pacing at a nationwide level for patients with SND. Methods and results.Using the administrative hospital database in France 2010-2020, 52,974 patients with SND were included in the analysis: 4,069 patients had VVI pacing and 48,905 had DDD pacing. Patients with leadless VVI pacemakers were excluded of the analysis. After propensity score matching 2,213 patients with VVI pacemaker were matched 1:1 with 2,213 patients treated with DDD pacemaker. In the matched analysis, patients with DDD pacemakers had a lower rate of all-cause (hazard ratio HR 0.711, 95%CI 0.61-0.828) and cardiovascular death (HR 0.628, 95%CI 0.48-0.818) within the 30 days after implantation. There were no significant differences for incidence of tamponade (HR 0.666, 95%CI 0.11-3.992), pneumothorax (HR 1.000, 95%CI 0.32-3.105), hemothorax (HR 0.800, 95%CI 0.21-2.982), major bleeding (HR 0.824, 95%CI 0.68-1.005) and transfusion (HR 1.016, 95%CI 0.83-1.243). During subsequent follow-up (mean: 3.0 ± 2.8 years), risk of all-cause death in the matched population was significantly lower in the DDD group than in the VVI pacemaker group (HR 0.683, 95%CI 0.60-0.784). Patients with SND treated DDD pacemakers also had a lower risk of cardiovascular death (HR 0.569, 95%CI 0.44-0.732), new-onset atrial fibrillation (HR 0.638, 95%CI 0.58-0.706), ischemic stroke (HR 0.685, 95%CI 0.53-0.887) and hospitalization for heart failure (HR 0.758, 95%CI 0.68-0.850) than those treated VVI pacemakers, whilst risk of endocarditis was not significantly different (HR 0.986, 95%CI 0.50-1.951).  Conclusion.Patients with SND treated with DDD pacemakers had better clinical outcomes compared to those treated with VVI pacemakers. DDD pacing was associated with lower risks of death, cardiovascular death, new-onset atrial fibrillation, ischemic stroke, hospitalization for heart failure. DDD pacing was neither associated with a higher risk of complication on the short-term nor of endocarditis on the longer-term.

scholarly journals De novo KCNQ1 mutation responsible for atrial fibrillation and short QT syndrome in utero

2005 ◽  
Vol 68 (3) ◽  
pp. 433-440 ◽  
Author(s):  
K HONG ◽  
D PIPER ◽  
A DIAZVALDECANTOS ◽  
J BRUGADA ◽  
A OLIVA ◽  
...  
Keyword(s):  
Atrial Fibrillation ◽  
De Novo ◽  
In Utero ◽  
Short Qt Syndrome ◽  
Qt Syndrome

scholarly journals Up to 6-year follow-up after pulmonary vein isolation for persistent/permanent atrial fibrillation: Importance of sinus node function

2011 ◽  
Vol 141 (6) ◽  
pp. 1455-1460 ◽  
Author(s):  
Yoshiharu Soga ◽  
Hitoshi Okabayashi ◽  
Yoshio Arai ◽  
Takuya Nomoto ◽  
Jota Nakano ◽  
...  
Keyword(s):  
Atrial Fibrillation ◽  
Pulmonary Vein ◽  
Pulmonary Vein Isolation ◽  
Sinus Node ◽  
Permanent Atrial Fibrillation

Atrial tachyarrhythmias in bradycardia–tachycardia syndrome: characterization and evolution

ESC CardioMed ◽  
2018 ◽  
pp. 1954-1957
Author(s):  
Luigi Padeletti ◽  
Roberto De Ponti
Keyword(s):  
Atrial Fibrillation ◽  
Atrial Flutter ◽  
Sinus Node ◽  
Ventricular Myocardium ◽  
Sinus Node Dysfunction ◽  
Ventricular Pacing ◽  
Atrial Tachyarrhythmia ◽  
Atrial Tachyarrhythmias ◽  
Node Disease ◽  
Sinus Node Disease

The association of sinus node disease and atrial tachyarrhythmias characterizes the bradycardia–tachycardia syndrome, which may result in an increased risk of heart failure, stroke, and death. Ageing and several cardiac and extracardiac diseases, which have the potential to affect both the atrial and the ventricular myocardium, can manifest their influence predominantly on the atria, leading to an atrial cardiomyopathy. In these cases, the same pathological process which leads to sinus node dysfunction can create a favourable substrate also for atrial tachyarrhythmias, which, if not present at the time of the initial diagnosis of the sinus node disease, can occur with an increasing prevalence during follow-up. In younger patients with no evident structural heart disease, a bradycardia–tachycardia syndrome may be the first clinical and unexpected manifestation of a still undiagnosed inherited genetic disease and therefore a specific diagnostic workup is necessary. In bradycardia–tachycardia syndrome, the most frequently encountered atrial tachyarrhythmia is atrial fibrillation, while typical atrial flutter is rarer. In peculiar subgroups of patients, other atrial tachyarrhythmias, such as atypical atrial flutter, macroreentrant or focal atrial tachycardia, may be present. In bradycardia–tachycardia syndrome, the evolution of atrial tachyarrhythmias clearly shows a worsening with an prevalence of associated atrial tachyarrhythmia over time. Pharmacological therapy for arrhythmias is of limited use, due to the concomitant sinus node dysfunction. The modality of pacing used to manage the sinus node disease has to be carefully chosen to minimize the evolution of atrial tachyarrhythmias. In fact, while ventricular pacing increases the incidence of atrial fibrillation and stroke, dual-chamber pacing with a specific algorithm for ventricular pacing minimization and prevention and treatment of atrial tachyarrhythmias reduces a composite endpoint of evolution to permanent atrial fibrillation, hospitalization, and death.

P1259Right atrial low voltage zone as a novel predictor of sinus node dysfunction in patients with non-paroxysmal atrial fibrillation

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
T Kondo ◽  
M Kimura ◽  
M Nakayama ◽  
O Matsuda
Keyword(s):  
Atrial Fibrillation ◽  
Low Voltage ◽  
Sinus Node ◽  
Pacemaker Implantation ◽  
Left Ventricular ◽  
Sinus Node Dysfunction ◽  
Right Atrial ◽  
Left Ventricular Ejection ◽  
Ablation Procedure ◽  
Ventricular Ejection Fraction

Abstract Background Although sinus node dysfunction (SND) coexists with atrial fibrillation (AF) in some cases, SND in patients with Non-paroxysmal AF (Non-PAF) could not be estimated in conventional electrophysiological study. Atrial low voltage zone (LVZ), which may be surrogate for atrial fibrosis, is although reported to present in patients with Non-PAF, the association between SND and right atrial LVZ (RA-LVZ) has not been fully evaluated. The aim of the present study was to assess the relationship between SND and RA-LVZ in patients with Non-PAF. Method Eighty-six Non-PAF patients underwent high density voltage mapping of right atrium (RA) during AF before ablation procedure. We defined LVZ as that with electrogram amplitude <0.1 mV in order to delineate strongly damaged area in RA. We evaluated the surface are of the RA-LVZ in Non-PAF patients with and without SND. Results Twenty-seven of 86 patients (31.4%) presented with SND after AF termination. There were no significant differences between patients with and without SND in variables such as age, sex, AF duration, left atrial diameter, and left ventricular ejection fraction. The mean value of RA-LVZ of all the patients was 12.1±11.4%, and RA-LVZ was significantly larger in patients with SND than in those without SND (22.8±14.6 vs 7.2±4.2%; P<0.001). In multivariate logistic regression analysis for the incidence of subsequent pacemaker implantation (PMI), only RA-LVZ was a significant predictor of subsequent PMI (odd ratio 1.306; 95% confidence interval 1.159 - 1.473; P<0.001). Receiving-operating characteristic curve for PMI following ablation procedure indicated cut-off value 10.5% for RA-LVZ with 85.2% sensitivity and 88.1% specificity (area under curve = 0.924, P<0.001). Kaplan-Meier analysis of the incidence of PMI after AF termination showed that freedom from pacemaker implantation was significantly better in patients with RA-LVA <10.5% than in those with RA-LVZ ≥10.5% (log-rank test; P<0.001). Conclusions Broad RA-LVZ measured during AF was strongly associated with SND and PMI after AF termination in patients with Non-PAF. Evaluation of RA-LVZ during AF could be a potential target in predicting SND requiring PMI in patients with Non-PAF.

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