Familial Atrial Standstill in Association with Dilated Cardiomyopathy

Fazelifar, Amir Farjam; Arya, Arash; Haghjoo, Majid; Sadr‐Ameli, Mohammad Ali

doi:10.1111/j.1540-8159.2005.00198.x

Cited by 23 publications

(16 citation statements)

References 16 publications

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“…Isolated atrial standstill was reported for the first time by Harley (5). Atrial standstill in association with dilated cardiomyopathy and ventricular tachycardia has been reported too (6). The cause of abnormality in this patient is unknown.…”

Section: Discussionmentioning

confidence: 99%

Persistent isolated right atrial standstill associated with left atrial tachycardia

2014

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Introduction:Atrial standstill is a rare condition, characterized by absence of atrial electrical and mechanical activity evident in surface electrocardiography echocardiography, or fluoroscopy, which is associated with unresponsiveness of atria to maximal output electrical stimulation. This condition can be present with thromboembolic complication, low cardiac output, and sometimes palpitation.Case Presentation:Here we presented a woman with right atrial stand still and left atrial tachycardia. It was confirmed by electrocardiogram, echocardiography, and intracardiac electrogram in basal state and during maximal output electrical stimulation. We treated her by implanting pacemaker to control bradycardia, oral calcium channel blocker to control palpitation episodes, and anticoagulation.Conclusions:Atrial standstill can be present partially that can be localized in one atrium and is associated with tachycardia in the other atrium.

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Section: Discussionmentioning

confidence: 99%

Persistent isolated right atrial standstill associated with left atrial tachycardia

2014

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“…Symptomatic cases require ventricular pacing and treatment for congestive heart failure if present, however, the prognosis is poor. Fazelifar et al1 reported a family with atrial standstill associated with syncope, dilated cardiomyopathy, and sudden cardiac death, and agreed with the postulation that the atrial standstill is a disease primarily associated with SCN5A mutation and rare connexin 40 genotype 5. Bhuiyan et al9 presented two families with typical CPVT in conjunction with additional features of progressive AV block, sinus node dysfunction, atrial fibrillation, atrial standstill, and dilated cardiomyopathy those related with the genetic defect in the N-terminus of RYR2.…”

Section: Discussionmentioning

confidence: 99%

“…On surface ECG, atrial standstill is distinguished by bradycardia, junctional (usually narrow complex) or wide complex escape rhythm, and absence of the P wave 1. Atrial standstill can be persistent or transient, and diffuse or partial.…”

Section: Introductionmentioning

confidence: 99%

Implantable Cardioverter-Defibrillator Implantation in a Patient with Atrial Standstill

et al. 2009

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We report a 55-year-old female patient who presented with no P waves but with a wide QRS complex escape rhythm at 44 beats/min and prolonged QTc of 0.55 seconds on ECG. The patient had recurrence of ventricular fibrillations and loss of consciousness, and underwent defibrillation and cardiopulmonary resuscitation (CPR) several times because of cardiac arrest. The transthoracic echocardiography showed dilated cardiomyopathy and enlargement of both atria. The Doppler echocardiography documented the absence of A wave in the tricuspid and mitral valve flow. An electrophysiologic study demonstrated electrical inactivity in the right and left atria. Atrial pacing with maximum output did not capture the atria. These findings together with her electrocardiographic finding indicated atrial standstill. Sudden cardiac death was her first clinical manifestation of ventricular arrhythmia. The patient remained asymptomatic after receiving a single chamber implantable cardioverter-defibrillator (ICD) with VVI pacemaker function.

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“…[1][2][3][4][5][6][7] There also may be an association with cardiomyopathy. [11][12][13] To our knowledge, the current case is the first detailed electrophysiological assessment of this condition.…”

Section: Discussionmentioning

confidence: 99%

“…However, an association with syncope and sudden death indicates that the syndrome may not always be benign. 1,11,30 This could result from inconsistencies in the junctional escape rhythm responsible …”

Section: Varma Et Al Sinus Node Dysfunction and Atrial Standstill E55mentioning

confidence: 99%

Congenital Sick Sinus Syndrome With Atrial Inexcitability and Coronary Sinus Flutter

Varma

Helms

Benson

et al. 2011

Circ: Arrhythmia and Electrophysiology

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ongenital sick sinus syndrome has been described sporadically. The syndrome may comprise various components of sinus bradycardia, atrial fibrillation, and right atrial inexcitability. [1][2][3][4][5][6][7] In the current case, detailed electrophysiological evaluation was undertaken with electroanatomic mapping of the right atrium and coronary sinus (CS) and of associated supraventricular arrhythmias. CaseA 37-year-old asymptomatic male physician from Mexico without any previous medical problems was referred after routine physical examination for evaluation of atrial fibrillation with a slow ventricular rate. Arrhythmia duration was unknown. He stated that bradycardia had been consistently noted on previous clinical examinations and had been attributed to competitive long-distance running since childhood. Heart rates in the "40s" had been noted since age 10 to 11 years when he had been informed of a "heart murmur." Subsequent echocardiographic examination had been normal. The patient denied any family history of sudden death or arrhythmias. He had no siblings and no children. His prior records (and those of his parents) were unavailable.Physical examination was normal except for a regular bradycardia of 45 beats/min. Twelve-lead ECG showed fixed R-R intervals. No clear P-wave activity was identified. However, there was small-amplitude baseline electric activity of debatable origin, interpreted variously by experienced electrocardiographers as standstill, artifact, or atrial fibrillation waves ( Figure 1A). A transthoracic echocardiogram showed normal ventricular volume and function (left ventricular end-diastolic diameter, 52 mm; left ventricle end-systolic diameter, 34 mm) and mild tricuspid regurgitation with estimated pulmonary artery systolic pressure of 40 mm Hg. Both atria were severely enlarged (left atrial volume index, 58 mL; right atrial area, 41 cm 2 ). Doppler echocardiographic examination of both tricuspid and mitral atrioventricular (AV) inflow showed complete absence of A waves, indicating lack of left and right atrial contractile activity (Figure 2). A transesophageal echocardiogram did not reveal any evidence of an intracardiac shunt. A Bruce protocol exercise treadmill test was stopped at 10 minutes and 26 s because of patient shortness of breath. Heart rate accelerated gradually to a maximum of 131 beats/min and always remained regular. Baseline atrial activity (if any) was not interpretable because of exercise artifact. Isolated premature ventricular contractions were observed during exercise. A 24-hour Holter monitor showed regular rhythm (mean heart rate, 47 beats/ min; range, 35-95 beats/min; maximum R-R interval, 1.8 s) with 56 ventricular bigeminal cycles. The only symptom during recording periods was an abnormal lateral chest sensation that corresponded with heart rates between 30 and 40 beats/min. The patient underwent an external cardioversion without appreciable change in heart rate (44 beats/min), although electrocardiographic intervals between QRS-T complexes became completely isoel...

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Familial Atrial Standstill in Association with Dilated Cardiomyopathy

Cited by 23 publications

References 16 publications

Persistent isolated right atrial standstill associated with left atrial tachycardia

Persistent isolated right atrial standstill associated with left atrial tachycardia

Implantable Cardioverter-Defibrillator Implantation in a Patient with Atrial Standstill

Congenital Sick Sinus Syndrome With Atrial Inexcitability and Coronary Sinus Flutter

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