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International Journal of Cardiology May 2013
Topics: Adult; Brain Ischemia; Cardiomyopathies; Electrocardiography; Female; Genetic Diseases, Inborn; Heart Atria; Heart Block; Humans; Stroke
PubMed: 23194782
DOI: 10.1016/j.ijcard.2012.10.077 -
JACC. Clinical Electrophysiology Jan 2023Atrial standstill (AS) is a rare condition characterized by absence of electrical activity within the atria. Studies to date have been limited.
BACKGROUND
Atrial standstill (AS) is a rare condition characterized by absence of electrical activity within the atria. Studies to date have been limited.
OBJECTIVES
The authors sought to describe the clinical characteristics, genetics, and outcomes of patients with AS.
METHODS
This was a retrospective multicenter study of patients <18 years at AS diagnosis, defined as absence of atrial activity documented during an electrophysiology study, device placement, or noninvasive rhythm tracings and confirmed by echocardiogram. Patients with acquired disorders were excluded. Clinical details and genetic variants were recorded and analyzed.
RESULTS
Twenty patients were diagnosed at a median age of 6.6 years (IQR: 2.9-10.8 years). Arrhythmias included 16 (80%) with atrial/supraventricular arrhythmias and 8 (40%) with ventricular tachycardia, including 4 with cardiac arrests. A type 1 Brugada pattern was documented in 4. Pacemakers were implanted in 18 (90%). Although atrial leads were attempted in 15, only 4 achieved pacing at implantation. During a median follow-up of 6.9 years (IQR: 1.2-13.3 years), 7 (35%) had thromboembolic events. Of these, none had atrial pacing, 6 were not on anticoagulation, and 1 was on aspirin. Genetic testing identified SCN5A variants in 13 patients (65%). Analyses suggest SCN5A loss-of-function may be one mechanism driving AS. Ventricular arrhythmias and cardiac arrest were more commonly seen in patients with biallelic SCN5A variants.
CONCLUSIONS
AS may be associated with loss-of-function SCN5A variants. Patients demonstrate atrial and ventricular arrhythmias, and may present challenges during device placement. Patients without the capacity for atrial pacing are at risk for thromboembolic events and warrant anticoagulation.
Topics: Humans; Child; Child, Preschool; Atrial Fibrillation; Heart Atria; Heart Block; Heart Arrest; Anticoagulants
PubMed: 36435694
DOI: 10.1016/j.jacep.2022.08.022 -
JACC. Clinical Electrophysiology Jan 2023
Topics: Humans; Child; Cardiomyopathies; Heart Atria; Heart Block
PubMed: 36697202
DOI: 10.1016/j.jacep.2022.11.025 -
Journal of the American Society of... Jul 2020
Topics: Atrial Fibrillation; Cardiomyopathies; Cardiomyopathy, Hypertrophic; Genetic Diseases, Inborn; Heart Atria; Heart Block; Humans; Interatrial Block
PubMed: 32359950
DOI: 10.1016/j.echo.2020.02.014 -
Ryoikibetsu Shokogun Shirizu 1996
Review
Topics: Arrhythmias, Cardiac; Atrial Function; Electrocardiography; Electrophysiology; Humans
PubMed: 9047480
DOI: No ID Found -
Anales de Pediatria Dec 2023
Topics: Humans; Cardiomyopathies; Heart Block; Heart Atria; Muscular Dystrophies
PubMed: 37949737
DOI: 10.1016/j.anpede.2023.11.001 -
Italian Heart Journal. Supplement :... Sep 2004Atrial standstill is a rare arrhythmogenic condition characterized by the absence of electrical and mechanical activity in the atria, transient or persistent, and... (Review)
Review
Atrial standstill is a rare arrhythmogenic condition characterized by the absence of electrical and mechanical activity in the atria, transient or persistent, and complete or partial. It can be "idiopathic", sporadic or familial, or secondary to Ebstein's anomaly, Emery-Dreifuss muscular dystrophy (X-linked), Kugelberg-Welander syndrome (autosomal recessive), and amyloidosis. Idiopathic familial atrial standstill is inherited as autosomal dominant trait with variable penetrance. To date, a few cases of familial forms of primary atrial standstill have been described. In each family, the number of affected members was small and limited to relatives of one generation. The genetic basis for familial atrial standstill is unknown. Recently a mutation in the cardiac sodium channel gene SCN5A associated with relatively rare genotypes for two connexin 40 polymorphisms has been reported. The diagnosis relies on the ECG demonstration of bradycardia, absence of P waves, and junctional narrow complex escape rhythm. Nearly 50% of patients suffer from Adams-Stokes attacks. In the primary persistent form, the atrial paralysis is paralleled by atrial dilation, mitral valve incompetence, and thrombotic complications, with high risk of thromboembolic complications. The treatment is addressed to the thromboembolic risk (anticoagulation), mitral incompetence (diuretics and vasodilators) and syncope (pacemaker implantation).
Topics: Adams-Stokes Syndrome; Anticoagulants; Arrhythmias, Cardiac; Bradycardia; Connexins; Diuretics; Electrocardiography; Heart Atria; Humans; Mitral Valve Insufficiency; NAV1.5 Voltage-Gated Sodium Channel; Pacemaker, Artificial; Prognosis; Risk Factors; Sodium Channels; Syncope; Thromboembolism; Vasodilator Agents; Gap Junction alpha-5 Protein
PubMed: 15568605
DOI: No ID Found -
SAGE Open Medical Case Reports 2023The case report shares evidence for a better understanding of atrial standstill. This being a rare arrhythmogenic condition. This is a 46-year-old woman presented with...
The case report shares evidence for a better understanding of atrial standstill. This being a rare arrhythmogenic condition. This is a 46-year-old woman presented with multiple sites of arterial embolism, including lower extremity arteries, coronary artery, and cerebral artery. Unexpectedly, multiple arterial embolization in the patient was due to atrial standstill by transthoracic echocardiography and cardiac electrophysiological study. An additional family investigation revealed that the patient's brother and sister also suffered from this disease. In search of further understanding the case, we carried out the genetic testing of the family and a frame shift double-G insertion mutation at c.1567 in the gene was found in all the three individuals. The patient recovered well after anticoagulation therapy and left bundle branch area pacing. This report remarks on the importance of multiple sites of arterial embolism which should be wary of family atrial standstill.
PubMed: 37425136
DOI: 10.1177/2050313X231179810 -
SAGE Open Medical Case Reports 2019Atrial standstill is a rare arrhythmia defined by the absence of mechanical and electrical activity in the atria. Few cases of atrial standstill have been described in...
Atrial standstill is a rare arrhythmia defined by the absence of mechanical and electrical activity in the atria. Few cases of atrial standstill have been described in children, none of which have presented with cerebral infarction confirmed by imaging. We report a unique case of a 7-year-old girl presenting with expressive aphasia, central facial palsy and irregular pulse with cerebral infarction secondary to atrial standstill. This case illustrates that cardiogenic cerebral embolism in children can be caused by rare conditions like atrial standstill and should be considered in paediatric patients undergoing evaluation for stroke. There are no established treatment guidelines for atrial standstill. We recommend that treatment be directed towards any potential underlying cause. All patients with atrial standstill should receive long-term oral anticoagulation treatment and a permanent cardiac pacemaker implant to reduce the risk of further strokes or other cardiac events.
PubMed: 30783526
DOI: 10.1177/2050313X19827735 -
The American Journal of Emergency... May 2016
Topics: Atrial Fibrillation; Cardiomyopathies; Electrocardiography; Heart Arrest; Heart Atria; Heart Block; Humans
PubMed: 26976773
DOI: 10.1016/j.ajem.2016.02.071