scholarly journals Severe congenital RYR1-associated myopathy complicated with atrial tachycardia and sinus node dysfunction: a case report

2019 ◽  
Vol 45 (1) ◽  
Author(s):  
Itaru Hayakawa ◽  
Yuichi Abe ◽  
Hiroshi Ono ◽  
Masaya Kubota
Keyword(s):  
Atrial Tachycardia ◽  
Heart Diseases ◽  
Sinus Node ◽  
Holter Monitoring ◽  
Sinus Node Dysfunction ◽  
Muscular Dystrophies ◽  
Strong Impact ◽  
Supraventricular Arrhythmia ◽  
Ectopic Atrial Tachycardia ◽  
Bulbar Paralysis

Abstract Background Cardiac arrhythmias are sometimes encountered in patients with hereditary myopathies and muscular dystrophies. Description of arrhythmias in myopathies and muscular dystrophies is very important, because arrhythmias have a strong impact on the outcomes for these patients and are potentially treatable. Case presentation A girl with severe congenital RYR1-related myopathy exhibited atrial tachycardia and sinus node dysfunction during infancy. She was born after uncomplicated caesarian delivery. She showed no breathing, complete ophthalmoplegia, complete bulbar paralysis, complete facial muscle paralysis, and extreme floppiness. At 5 months old, she developed persistent tachycardia around 200–210 beats per minutes. Holter monitoring revealed ectopic atrial tachycardia during tachyarrhythmia and occasional sinus pauses with junctional escape beats. Propranolol effectively alleviated tachyarrhythmia but was discontinued due to increased frequency and duration of the sinus pauses that led to bradyarrhythmia. There was no evidence of structural heart diseases or heart failure. The arrhythmia gradually resolved spontaneously and at 11 months old, she showed complete sinus rhythm. Conclusions Although supraventricular arrhythmia is sometimes encountered in congenital myopathies, this is the first report of cardiac arrhythmia requiring drug intervention in RYR1-associated myopathy.

Focal (ectopic) atrial tachycardia

2018 ◽  
Author(s):  
Michael Jones ◽  
Norman Qureshi ◽  
Kim Rajappan
Keyword(s):  
Atrial Tachycardia ◽  
Sinus Node ◽  
P Wave ◽  
Ventricular Rate ◽  
Av Block ◽  
Ectopic Atrial Tachycardia ◽  
Wave Morphology ◽  
Focal Atrial Tachycardia ◽  
The Difference ◽  
The Right

Focal atrial tachycardia is an atrial arrhythmia arising in either the left or the right atrium, usually faster than 100 min−1 and regular, with a P-wave morphology that is different from the normal P-wave morphology associated with sinus rhythm—the difference in morphology being more pronounced the further away the focus lies from the sinus node. The ventricular rate is generally fast also, dependent on the nature of the atrioventricular conduction (AV); 1:1 conduction may be seen, especially in younger patients or patients with accessory pathways capable of very rapid antegrade conduction; alternatively, 2:1, Wenckebach-type, or higher-grade AV block may be seen.

scholarly journals Syncope due to sinus node dysfunction after surgical patch closure of atrial septal defect: A case report

2017 ◽  
Vol 70 (5-6) ◽  
pp. 167-169
Author(s):  
Larisa Dizdarevic-Hudic ◽  
Zumreta Kusljugic ◽  
Irma Bijedic ◽  
Igor Hudic
Keyword(s):  
Case Report ◽  
Atrial Septal Defect ◽  
Septal Defect ◽  
Sinus Node ◽  
Pacemaker Implantation ◽  
Holter Monitoring ◽  
Sinus Node Dysfunction ◽  
Surgical Closure ◽  
Patch Closure ◽  
Possible Cause

Introduction. Sick sinus syndrome, a frequent cause of syncope, refers to a combination of symptoms caused by sinus node dysfunction. Case report. We report the case of a 38-year-old female patient presenting with recurrent syncope, who underwent surgical patch closure of atrial septal defect three years before admission. Ambulatory twenty-four-hour Holter monitoring was done capturing only sinus tachycardia. A series of examinations were warranted after admission, and recurrent syncope was found to be the result of sinus node dysfunction. This syndrome rarely occurs after surgical closure of atrial septal defect. The patient underwent permanent pacemaker implantation. Conclusion. A rigorous search for every possible cause of syncope is mandatory. A structural, multidisciplinary approach is required in order to achieve an optimal outcome.

P957Characteristics of atrial tachycardia after mitral surgey via biatrial trans-septal approach using high density mapping system

EP Europace ◽  
2020 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
C Cataldi ◽  
M Andronache ◽  
R Eschalier ◽  
F Jean ◽  
R Bosle ◽  
...  
Keyword(s):  
Atrial Tachycardia ◽  
Sinus Node ◽  
Pulmonary Veins ◽  
High Density ◽  
Congenital Defects ◽  
Density Mapping ◽  
Mapping System ◽  
Oblique Incision ◽  
Pace Maker ◽  
The Right

Abstract Background The biatrial trans-septal approach (BTSa) ameliorates mitral valve (MV) exposure in difficult cases when routine left atriotomy doesnt"t allow it. Main steps are an oblique incision on the right atrium (RA), reaching medially the right pulmonary veins (PV), a septal incision from the fossa ovalis, extended up to reach the first incision, then on the left atrium (LA). Purpose We aim to study the arrhythmic burden in this post-surgical context, focusing on atrial tachycardia (AT), to investigate the complexity of several possible circuits. Methods All patients (>18yo) with previous MV surgery via BTSa for MV repair or replacement, who underwent ablation of AT from January 2017 to September 2019, were enrolled. Patients ablated for persistent or paroxysmal AF, or with AF during the index procedure were excluded. Patients with associated surgery on other valves or congenital defects, coronary, surgical or percutaneous rhythm interventions weren’t excluded. Electroanatomical mapping was created using 2 different high-density mapping system. Substrate and activation map and radio-frequency (RF) ablation (25-50W, Ablation Index target 400) were realized. Cartographies were analysed to evaluate AT re-entry circuit, critical isthmus (CI) location and characterization, atrial vulnerability. Procedural outcomes (AT termination, sinus rhythm (SR) restoration, anti-arrhythmic drugs (AAD) withdrawal), and peri-procedural complications were also evaluated. Results We enrolled 49 patients (median age 57 ± 15), finding a maximum of 5 AT per procedure (2 ± 1). A total of 112 AT were mapped: the majority (72%) were persistent AT, 8,2% common atrial flutter. Cycle length was 314 ± 74 msec, with proximal-distal activation of coronary sinus (78%). A multiple re-entry circuit was observed in 70% of index AT. We identified 152 critical isthmus (maximum 5 per procedure). Only 27,9% of our patients had a single CI; CTI was the most frequent one (n = 37), envolved in 33% of all AT, while BTS scars altogether were envolved in 65% AT. A complete AT circuit was mapped in the RA, the LA and both atria in respectively 49%, 11,5% and 39%AT. The distribution of CIs is shown in figure 1. Biatrial and left AT leads to superior procedure, RF and fluoroscopy duration (p <0,05). SR was restored in 93,4%of patients, requiring a DC shock in 4 cases. Immediate AAD withdrawal was achieved after 41%procedures. No pericardial, oesophageal, vascular or phrenic complication occurred. 4 pace-maker implantations were realized because of 3 interatrial, 2 AV block and a sinus node dysfunction. Conclusions AT occurring after a BTSa have a high prevalence of multiple re-entry circuits with multiple critical isthmus. Ablation in this context is feasible and safe but often requires a left atrial access. Mapping of both atria should be considered to identify critical isthmus and tailored ablation strategy. Abstract Figure 1. Critical Isthmus Distribution

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