Permanent atrial standstill: The clinical spectrum

Woolliscroft, James O.; Tuna, Naip

doi:10.1016/0002-9149(82)90226-0

Cited by 54 publications

(39 citation statements)

References 25 publications

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“…The progressive nature of the disease has been reported previously. In some families without evidence of underlying disease, symptoms were first noted as early as age 1 to 3.5 years 6,9 or in mid-teens, 1,8 whereas in other families the onset of the disease was in the late twenties or thirties, 7,10 as is the case in the family reported here.…”

Section: Comparison With Other As Familiessupporting

confidence: 53%

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A Cardiac Sodium Channel Mutation Cosegregates With a Rare Connexin40 Genotype in Familial Atrial Standstill

Groenewegen

Firouzi

Bezzina

et al. 2003

Circulation Research

259

221

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Abstract-Atrial standstill (AS) is a rare arrhythmia that occasionally appears to be genetically determined. This study investigates the genetic background of this arrhythmogenic disorder in a large family. Forty-four family members were clinically evaluated. One deceased and three living relatives were unambiguously affected by AS. All other relatives appeared unaffected. Candidate gene screening revealed a novel mutation in the cardiac sodium channel gene SCN5A (D1275N) in all three affected living relatives and in five unaffected relatives, and the deceased relative was an obligate carrier. In addition, two closely linked polymorphisms were detected within regulatory regions of the gene for the atrial-specific gap junction protein connexin40 (Cx40) at nucleotides Ϫ44 (G3 A) and ϩ71 (A3 G). Eight relatives were homozygous for both polymorphisms, which occurred in only Ϸ7% of control subjects, and three of these relatives were affected by AS. The three living AS patients exclusively coinherited both the rare Cx40 genotype and the SCN5A-D1275N mutation. SCN5A-D1275N channels showed a small depolarizing shift in activation compared with wild-type channels. Rare Cx40 genotype reporter gene analysis showed a reduction in reporter gene expression compared with the more common Cx40 genotype. In this study, familial AS was associated with the concurrence of a cardiac sodium channel mutation and rare polymorphisms in the atrial-specific Cx40 gene. We propose that, although the functional effect of each genetic change is relatively benign, the combined effect of genetic changes eventually progresses to total AS. (Circ Res. 2003;92:14-22.)

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Section: Comparison With Other As Familiessupporting

confidence: 53%

“…1,2 Electrocardiographically, AS is characterized by bradycardia, the absence of P waves, and a junctional narrow complex escape rhythm. Different forms of the arrhythmia have been described, such as persistent versus transient AS 3 and a total versus a partial form.…”

mentioning

confidence: 99%

A Cardiac Sodium Channel Mutation Cosegregates With a Rare Connexin40 Genotype in Familial Atrial Standstill

Groenewegen

Firouzi

Bezzina

et al. 2003

Circulation Research

259

221

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“…SSS is diagnosed electrocardiographically and can have variable presentations, ranging from sinus bradycardia, sinus arrest, atrial standstill, and tachycardia‐bradycardia syndrome. Atrial standstill is a rare condition that is characterized by the absence of electrical and mechanical atrial activity . The electrocardiogram (ECG) is characterized by absent P waves and a narrow complex escape rhythm.…”

Section: Introductionmentioning

confidence: 99%

A homozygous SCN5A mutation associated with atrial standstill and sudden death

Tan

Gando

et al. 2018

Pacing Clinical Electrophis

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Section: Introductionmentioning

confidence: 99%

“…Atrial standstill is a rare heterogeneous arrhythmia characterized by electrical and mechanical standstill and electrical inexcitability 1–4 . A long‐lasting progressive form is seen with cardiac 3–6 and neuromuscular diseases 7–10 . Previously described familial 11 and idiopathic 12 forms may be due to coinheritance of a SCN5A mutation and connexin 40 polymorphism 13 .…”

Section: Introductionmentioning

confidence: 99%

Prolonged Transient Atrial Electrical Silence Following Termination of Chronic Atrial Tachyarrhythmias

Turitto

Saponieri

Onuora

et al. 2007

Pacing Clinical Electrophis

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The present report expands the spectrum of the syndrome of atrial standstill and raises interesting questions regarding possible electrophysiologic mechanism(s) of prolonged post overdrive atrial standstill. The report suggests that chronic overdrive of sinus and subsidiary atrial pacemakers may result in calcium overloading of cardiac cells, which is known to cause suppression of pacemaker activity as well as increased intracellular resistance. These mechanisms can possibly result in either prolonged suppression of sinus and atrial pacemaker activity and/or pacemaker exit block.

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Permanent atrial standstill: The clinical spectrum

Cited by 54 publications

References 25 publications

A Cardiac Sodium Channel Mutation Cosegregates With a Rare Connexin40 Genotype in Familial Atrial Standstill

A Cardiac Sodium Channel Mutation Cosegregates With a Rare Connexin40 Genotype in Familial Atrial Standstill

A homozygous SCN5A mutation associated with atrial standstill and sudden death

Prolonged Transient Atrial Electrical Silence Following Termination of Chronic Atrial Tachyarrhythmias

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