ArticleJACC. Clinical electrophysiology2023
Atrial Standstill in the Pediatric Population: A Multi-Institution Collaboration.
Article in JACC. Clinical electrophysiology, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers, 1 of them a synthesis that pooled it.
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13 citing papers in PubMed, 1 synthesis or guideline pooled it, 16 citations in OpenAlex.
- Diagnosis and management of very rare primary arrhythmia syndromes in children and adults: a Clinical Consensus Statement of the European Heart Rhythm Association of the ESC and the Association of Cardiovascular Nursing & Allied Professions of the ESC, endorsed by the Association for European Paediatric and Congenital Cardiology.Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology · 2026Guideline
- Brugada syndrome in a boy aged 15 months.HeartRhythm case reports · 2026Article
- A cardiac sodium channelopathy overlap syndrome presenting as stroke in a young child.HeartRhythm case reports · 2026Article
- The Arrhythmic Burden of Cardiac Amyloidosis: A Comprehensive Review.Heart views : the official journal of the Gulf Heart Association · 2026Review
- Atrial Dilated Cardiomyopathy: From Molecular Pathogenesis to Clinical Implications.Journal of clinical medicine · 2025Review
- Clinical presentation and outcomes of patients with biallelicHeart rhythm O2 · 2025Article
- Cardio-Embolic Stroke in a 14-Year-Old Boy Due to SCN5A-Associated Atrial Standstill.JACC. Case reports · 2025Article
- A Novel SCN5A Mutation (c.589G>A) Is Associated With Intermittent Atrial Standstill and Conduction System Disease.JACC. Case reports · 2025Article
- A Case of Atrial Standstill Misdiagnosed as Atrial Fibrillation: Diagnostic Insights and Clinical Implications.JACC. Case reports · 2025Article
- Atrial standstill due to eosinophilic myocarditis and drug reaction with eosinophilia and systemic symptoms.HeartRhythm case reports · 2025Article
- Episodic long-lasting atrial standstill associated with anHeartRhythm case reports · 2025Article
- Atrial standstill in a young patient with ischemic stroke associated with inheritance of a novel HCN4 mutation.Journal of arrhythmia · 2024Article
- The Year in Pediatric Electrophysiology: 2023.The Journal of innovations in cardiac rhythm management · 2024Article
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Authors and funding
19 authors at 12 institutions in 3 countries.
Funding
Abstract
backgroundAtrial standstill (AS) is a rare condition characterized by absence of electrical activity within the atria. Studies to date have been limited.
objectivesThe authors sought to describe the clinical characteristics, genetics, and outcomes of patients with AS.
methodsThis 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.
resultsTwenty 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.
conclusionsAS 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.
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