scholarly journals Familial Sinus Node Disease Caused by a Gain of GIRK (G-Protein Activated Inwardly Rectifying K + Channel) Channel Function

Author(s):  
Johanna Kuß ◽  
Birgit Stallmeyer ◽  
Matthias Goldstein ◽  
Susanne Rinné ◽  
Christiane Pees ◽  
...  
2021 ◽  
Vol 12 (1) ◽  
pp. 70-75
Author(s):  
Anne Kathrine M. Nielsen ◽  
Vibeke E. Hjortdal

Background: Surgical repair of partial anomalous pulmonary venous connection (PAPVC) may disturb the electrical conduction in the atria. This study documents long-term outcomes, including the late occurrence of atrial tachyarrhythmia and bradyarrhythmia. Methods: This retrospective study covers all PAPVC operations at Aarhus University Hospital between 1970 and 2010. Outcome measures were arrhythmias, sinus node disease, pacemaker implantation, pathway stenosis (pulmonary vein(s), intra-atrial pathway, and/or superior vena cava), and mortality. Data were collected from databases, surgical protocols, and hospital records until May 2018. Results: A total of 83 patients were included with a postoperative follow-up period up to 46 years. Average age at follow-up was 43 ± 21 years. During follow-up, new-onset atrial fibrillation or atrial flutter appeared in four patients (5%). Sinus node disease was present in nine patients (11%). A permanent pacemaker was implanted in seven patients (8%) at an average of 12.7 years after surgery. Pulmonary venous and/or superior vena cava obstruction was seen in five patients (6%). Stenosis was most prevalent in the two-patch technique, and arrhythmia was most prevalent in the single-patch technique. Sixty-seven (81%) of 83 patients had neither bradyarrhythmias nor tachyarrhythmias or pacemaker need. Conclusions: This study contributes important long-term data concerning the course of patients who have undergone repair of PAPVC. It confirms that PAPVC can be operated with low postoperative morbidity. However, late-onset stenosis, bradyarrhythmias and tachyarrhythmias, and need for pacemaker call for continued follow-up.


2016 ◽  
Author(s):  
Inna Leybell ◽  
Liliya Abrukin

Bradyarrhythmias can present as an incidental electrocardiographic (ECG) finding or a life-threatening condition requiring immediate intervention. They are caused by sinus node disease or atrioventricular block. This review covers pathophysiology, stabilization and assessment, diagnosis and treatment options, and disposition and outcomes for patients with bradycardia. Figures in the review demonstrate characteristic ECG tracings. Tables list classifications of sinus node dysfunction as well as of sinoatrial and atrioventricular blocks, presenting symptoms, important information to elicit on history taking, various causes of bradycardia, and specific interventions for toxicologic etiologies. Key words: atrioventricular block, atropine, bradyarrhythmia, bradycardia, first-degree atrioventricular block, second-degree atrioventricular block, sinoatrial block, sinus bradycardia, sinus node disease, tachycardia-bradycardia syndrome, third-degree atrioventricular block This review contains 9 highly rendered figures, 7 tables, and 92 references.


2006 ◽  
Vol 9 (3) ◽  
pp. A123
Author(s):  
P Rucinski ◽  
A Kutarski ◽  
M Latek ◽  
B Kaminski ◽  
A Rubaj ◽  
...  

2003 ◽  
Vol 41 (6) ◽  
pp. 121 ◽  
Author(s):  
Prashanthan Sanders ◽  
Joseph B. Morton ◽  
Peter M. Kistler ◽  
Neil C. Davidson ◽  
Azlan Hussin ◽  
...  

ESC CardioMed ◽  
2018 ◽  
pp. 1954-1957
Author(s):  
Luigi Padeletti ◽  
Roberto De Ponti

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.


ESC CardioMed ◽  
2018 ◽  
pp. 1952-1953
Author(s):  
Dominic A. M. J. Theuns

Electrophysiological studies are indicated in patients with symptoms compatible with sinus node disease and in whom no documentation of the arrhythmia responsible for these symptoms has been obtained by prolonged electrocardiographic monitoring (e.g. Holter recording). In these patients, electrophysiological testing can yield information that may be used to guide appropriate therapy. The most useful measures of the overall sinus node function are the sinus node recovery time and the sinoatrial conduction time. In practice, the sinoatrial conduction time is rarely assessed because it represents an indirect measurement.


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